Metastatic melanoma of the ampulla of Vater is rare. The purpose of this study was to summarize the characteristics and outcomes of metastatic melanoma in the ampulla of Vater and highlight the impact of surgery on the prognosis of patients with metastatic melanoma. Pooled data from a case encountered at our institution and from all sporadic cases published on PubMed and MEDLINE between 1996 and 2023 were analyzed. Fourteen patients with metastatic melanoma in the ampulla of Vater were enrolled. Seventy-three percent of primary melanomas were cutaneous and 27% were mucosal. Jaundice was the most common symptom (86%). The size of metastatic melanoma to the ampulla ranged from 1.5 cm to 8 cm, with a median of 2.75 cm. Concomitant metastasis to other organs occurred in 82% of the patients at the time of diagnosis, most commonly in the brain, lungs, and liver (36% each). Among the reported cases, pancreaticoduodenectomy was performed in five patients. The overall 1-year survival rate was 27.3%, with a median survival of four months. Wide excision of the primary lesion and chemotherapy significantly improved survival rates (p= 0.048). There is a trend toward improved survival in patients undergoing pancreaticoduodenectomy followed by chemotherapy. Given the availability of effective systemic therapies, metastatic melanoma of the ampulla of Vater does not necessarily preclude major surgeries.