The carcinoid tumor of the ampulla of Vater is extremely rare. We report a case of 57-year old male with carcinoid tumor of ampulla of Vater. This patient had been presented with bronchial asthma for one year. Abdominal CT finding revealed diffuse dilatation of common bile duct and pancreatic duct, and also ampullary soft mass protruded into duodenal lumen. The correct diagnosis was made preoperatively by endoscopic retrograde cholangiography with biopsy. Pancreatico- duodenectomy with lymph nodes dissection was performed. Malignant carcinoid tumor originating from ampulla of Vater was diagnosed with pericholedochal lymphatic metastasis. Surgical removal led to complete resolution of asthmatic symptoms.(Korean J Med 59:220-224, 2000)