We report a case of vipoma of the pancreas in conjunction with ulcerative colitis in a 20-yr-old woman. Twenty months after the onset of ulcerative colitis, the patient complained of watery diarrhea and was found to have an electrolyte disorder. A pancreatic tumor was detected by ultrasonography and computed tomography, along with an elevation of serum vasoactive intestinal polypeptide (VIP). The secretory diarrhea diminished dramatically, and the serum VIP level decreased into the normal range immediately after resection of the pancreatic tumor. Immunohistochemical staining revealed a pancreatic vipoma. Despite removal of the vipoma, she underwent restorative proctocolectomy and ileal J-pouch anal anastomosis 2 yr later due to progression of the ulcerative colitis. Postoperative course was uneventful, with excellent functional results. Knowledge of this rare condition accompanying ulcerative colitis may help in the management of the patient with an atypical clinical course.