Background: Somatostatinomas of the duodenum are often associated with neurofibromatosis. The pancreas is a rather more frequent site for this type of neuroendocrine tumor, and most of these patients have somatostatinoma syndrome. A rare case of pancreatic somatostatinoma is reported in association with Kaposi's sarcoma in the resection specimen. Case outline: A 58-year-old man who had multiple cutaneous neurofibromas developed mild obstructive jaundice and was found to have three cystic masses in the head of pancreas on abdominal CT. Pylorus-preserving proximal pancreatoduodenectomy was performed, and pathologic examination revealed a 3.5-cm diameter somatostatinoma plus a focus of Kaposi's sarcoma in the head of pancreas. Stromal tumor was found in the duodenal wall, and two mucosal plaques of Kaposi's sarcoma were found in the gallbladder. Discussion: The association of somatostatinoma - itself one of the rarest types of neuroendocrine tumor - with Karposi's sarcoma and neurofibromatosis has not been reported previously. Of additional note is the fact that this patient was neither immunosuppressed nor positive for human immunodeficiency virus.