Acinar cell carcinoma is an uncommon malignancy with a reported incidence of 1% among exocrine tumors of the pancreas. The case of a 60-year-old Taiwanese man who presented with obstructive jaundice, abdominal pain, and body weight loss is described here. A mixed clinical picture of islet cell tumor and ductal carcinoma of the pancreas was shown to be a hypervascular tumor at the pancreatic head region with an irregular stricture at the common channel of the common bile and pancreatic ducts. The patient had normal levels of plasma carcinoembryonic antigen, carbohydrate antigen 19-9, alpha-fetoprotein, but an increase in plasma levels of insulin and C-peptide. Immunohistochemical stains and electron microscopic examination of the tumor was consistent with acinar cell carcinoma.