A 67-year-old woman presented with a chief complaint of malaise. CT showed a pancreatic head tumor 5 cm in diameter with calcification. It also showed multiple liver metastases and bile duct dilatation. Esophagogastroduodenoscopy showed deformation and stenosis on the superior and inferior side of the duodenal angle due to a papillary tumor. Thus, retrograde insertion of an endoscope was difficult, and a diagnosis of a carcinoid tumor was obtained by biopsy. After disclosing the diagnosis to the patient, we explained the necessity of treatment. However, the patient refused all proposed treatments, and therefore, we merely provided treatment of her symptom. The patient died of liver failure approximately 3 months later. At autopsy, multiple neurofibromas and café-au-lait spots were observed on the body surface. The tumor was a carcinoid tumor with distant metastases which originated from the duodenal papillary area. On histopathological examination, there were psammoma bodies in neoplastic cells and production of somatostatin. There were also stromal amyloid deposits. We report here a valuable case of carcinoid tumor with type 1 neurofibromatosis accompanied with amyloid deposits.