A 52-year-old woman presented with relapsing acute pancreatitis. A contrast CT scan revealed polysplenia, agenesis of the dorsal pancreas, preduodenal portal vein, inferior vena cava with persistent continuity of the azygos vein, abnormal lung lobation with bilateral left bronchial morphology, and intestinal malrotation (non-rotation type). To the best of our knowledge, this is the first report in which successful pancreatic duct stent placement for the treatment of recurrent pancreatitis was performed in a polysplenia patient with agenesis of the dorsal pancreas, separate bile and pancreatic ducts and Peutz-Jeghers syndrome.