Papillary stenosis is characterized by fixed fibrosis leading to structural outflow obstruction and it is usually secondary to inflammation and fibrosis from the chronic passage of gallstones, episodes of acute pancreatitis, chronic pancreatitis, sclerosing cholangitis, peptic ulcer disease, and cholesterolosis. However, obstructive jaundice with or without acute cholangitis which leads the physician to suspect the presence of malignancy as a cause is a rare manifestation of papillary stenosis. We report here a case of papillary stenosis presenting with obstructive jaundice and acute cholangitis. The lesion was so difficult to exclude the presence of malignancy preoperatively and intraoperatively that a pylorus-preserving pancreaticoduodenectomy was performed. Histologic examination of the resected specimen revealed fibrosis, adenomatoid ductal hyperplasia, and mild chronic inflammation of the papilla of Vater and distal common bile duct.