Merkel cell carcinoma (MCC) is a rare cutaneous malignancy thought to be derived from neuroendocrine cells. It is usually found in elderly men on the head, neck and extremities, and appears as a reddish-purple, non-tender nodule. Most often it presents as localized disease, but not infrequently it manifests regional node involvement and metastasizes distantly. Immunosuppression as a result of disease (chronic lymphocytic leukemia) or iatrogenic (organ transplantation) results in an increased frequency of MCC. We recently encountered a middle-aged male who was HIV-1 infected and manifested MCC on the dorsum of the hand. The patient is a 57-year-old podiatrist who has been HIV-1 positive for 14 years. He has never experienced an opportunistic infection and his CD4 cell counts have ranged from 357 to 573 cells/μl during the past 3 years. His viral load is consistently undetectable. In April 2002, he was found to have a 7 mm reddish-purple nodule on the dorsum of his right middle finger. A biopsy revealed MCC, and a wide excision was undertaken without further therapy. By June 2003, the tumor had recurred and now measured 1.5 × 1.5 cm on the dorsum of the right hand. Slightly firm and non-tender right-sided epitrochlear and axillary nodes measuring 0.5 × 0.5 cm were palpated. A positron emission tomography scan displayed slightly increased activity at these nodal sights. A computerized tomography scan of the chest, abdomen and pelvis as well as a bone scan were unremarkable. A complete blood count, chemistries, urinalysis and thyroid studies were normal but the neurone-specific enolase (NSE) was elevated to 19 (normal < 10). Moh's micrographic resection of the tumor with ultimately clear margins ensued. A previous lymphoscintigraphy and sentinal node biopsy failed to demonstrate metastatic nodal disease. He received radiotherapy to 6000 rads at the tumor sight. In June 2004, he developed ascites and paracentesis revealed a transudate with negative cytology and cultures including fungi and acid fast bacilli. Computerized tomography scans of the chest, abdomen and pelvis were unremarkable other than for the ascites. Laboratory studies revealed a slight elevation of the transaminses and alkaline phosphatase and a mild normocytic, normochromic anemia. Hepatitis A, B and C antibodies were undetected but the NSE remained elevated. A liver biopsy revealed moderate fibrosis without infiltrates, granulomata or neoplasm. It was decided to initiate combination chemotherapy despite the lack of cytological confirmation of neoplasia with carboplatin and etoposide, which he received for six cycles every 3 weeks. The patient is now more than a year post-chemotherapy and 40 months since the initial diagnosis of MCC with no evidence of disease and a normal NSE. MCC has only been reported in seven patients infected by HIV-1 [1–6] before this case report. However, a recent review by Engels et al. [7], using linked AIDS and cancer registries encompasing 309 365 patients, identified six cases of MCC corresponding to a relative risk of 13.4 (95% confidence interval 4.9–29.1) compared with the general population. However, the study could not examine whether MCC increased over time relative to AIDS onset or with CD4 lymphocyte depletion and HIV-1 viral load. Of the eight HIV-1 patients we reviewed with MCC their mean age was 48 years (36–60 years), all but one was male, and the woman was from Africa. In the United States, more than 85% of MCC patients uninfected by HIV-1 are men over the age of 60 years, whereas in patients with concomitant HIV-1 infection only one out of eight was at least 60 years of age. The mean time of established HIV-1 infection to a diagnosis of MCC was reported in six patients, and ranged from 3 to 15 years in five, and was simultaneous in one. Four out of six were receiving HAART therapy. The HIV-1 viral load ranged from 0 to 750 000 copies/ml3 in six patients in whom it was recorded. In one patient it was 750,000 copies/ml3, in two it was undetectable, and in the remainder ranged from 11 000 to 32 000 copies/ml3. The mean CD4 cell count was 309 cells/μl. In four out of eight patients the MCC was found on the buttock or in the groin, in three on the face or neck, and in our patient on the hand. In four individuals the initial lesion was associated with regional node involvement. None of the eight patients had evidence of systemic metastases at the time that MCC was discovered. Surgical excision of the MCC was accomplished in all patients and six received local or regional radiation therapy. None received adjuvant chemotherapy, but three were given chemotherapy for confirmed or presumed metastatic disease and one patient was not. Three of the four latter patients died. With the persistence of HIV disease one would anticipate increasing numbers of MCC, necessitating a study of the role of adjuvant chemotherapy in preventing metastases.