We report here the first case of choledochal cyst associated with a benign stenosis of the cephalic part of the main pancreatic duct. The pancreatic ductal stenosis was associated with a protein plug located upstream of the stenosis. Preoperatively, it was not possible to rule out a localized intraductal pancreatic tumor, and a pylorus-preserving pancreaticoduodenectomy was performed. This association has not been described previously, and gives new insights into the pathogenesis of acute pancreatitis associated with choledochal cyst.