INTRODUCTION: Kaposi sarcoma (KS) is a low grade angioproliferative tumor. AIDS-related KS is the most common and aggressive form of KS. The prevalence of AIDS-related KS is up to 30%. Skin is the most commonly affected organ in 78% of cases, The gastrointestinal (GI) tract is the most commonly affected extracutaneous site, and often asymptomatic. In advanced cases, patients may present with GI bleeding, diarrhea, protein-losing enteropathy, intussusception, or perforation. Although only 20% of KS patients experience GI symptoms, some studies have found that up to 80% of KS patients have GI lesions at autopsy. With the advent availability of HARRT therapy, the incidence of Kaposi sarcoma has decreased dramatically. Our objective is to present an unusual presentation of Kaposi sarcoma and its endoscopic findings leading to the diagnosis of KS, to share how endoscopy can be used as a tool for diagnosing and assessing the GI involvement in KS. CASE DESCRIPTION/METHODS: A 35-year-old African American male with HIV, syphilis, and hepatitis B complains of swelling of his legs and scrotum for 2 months. Physical examination revealed abdominal distension, bilateral lower extremity edema, and one skin nodule on his right foot. Laboratory studies revealed Hgb 8.7 g/dL, MCV 95.3 fL/cell, WBC 7500/cmm, CD4 count < 80 cells/uL, and albumin 1.4 g/dL. CT imaging revealed significant abdominal ascites (Figure 1). The patient underwent paracentesis removing 3.5 L of milky fluid. Fluid culture, gram stain and cytology were negative. Given the ascites and hepatitis history, an esophagogastroduodenoscopy (EGD) was completed in evaluating for possible esophageal varices. EGD revealed a nodular fungating lesion that extends diffusely from the gastric body into the duodenal bulb and sweep (Figures 2 and 3). Biopsy revealed cylindrical cells positive for HHV-8, confirming the diagnosis of gastric Kaposi's sarcoma (KS). Immunohistochemical analysis of the skin nodule biopsy confirmed KS. Computed tomography scan of the chest was consistent with pulmonary metastases. Patient is being treated with HAART and will be started on chemotherapy. DISCUSSION: KS is a rare neoplasm, and an AIDS-defining illness in HIV patients. About 40-51% of HIV patients with cutaneous KS will develop gastrointestinal KS, which is typically asymptomatic until growth is extensive enough to cause abdominal pain. This case illustrates the importance of endoscopic evaluation in HIV patients for early detection of gastrointestinal involvement of KS.