Cystadenomas and cystadenocarcinomas are the most common cystic tumors of the pancreas. Their inner lining contains neuroendocrine cells that may, in rare cases, produce a systemic syndrome. Similarly, digestive or pancreatic endocrine tumors have been reported in association with cystic tumors of the pancreas. We report a case of cystadenocarcinoma of the pancreas head, associated with a serous cystadenoma and an endocrine tumor of the body of the pancreas. In a 70 year old patient, jaundiced due to obstruction of the extra-hepatic bile ducts, the abdominal ultrasound and CT scan showed a hypoechogenic hypodense lesion of the pancreas head resulting in an enlargement of the common bile duct and pancreatic duct. Echoendoscopy confirmed the diagnosis and gave additional information; it showed that the cephalic lesion was heterogeneous, with anechogene spaces separated by coarse echogenic septae associated to a solid contingent. Also detected by echoendoscopy was a cystic lesion containing thin septae and a contiguous hypoechogenic nodule of the body of the pancreas. Histology of the specimen following total pancreatectomy revealed a cephalic cystadenocarcinoma associated with a serous cystadenoma and a non-secreting endocrine tumor of the body region. This observation highlights the usefulness of echoendoscopy in the work-up of cystic lesions of the pancreas; indeed, the tumors of the body of the pancreas were not detected by others methods. Furthermore, there are reports in literature on endocrine secretion syndromes secondary to digestive endocrine tumors or neuroendocrine cells in the lining of cystic tumors of the pancreas.