Hydatid cyst of the pancreas is a rarely seen entity even in endemic countries. Cyst may causes several symptoms due to external compression or fistulisation to pancreaticobiliary tract or small bowel. A 23-year-old female patient was referred with a complaint of abdominal pain. Preoperative imaging revealed an undefined cyst in the tail of pancreas. She underwent distal pancreatectomy and splenectomy, with a diagnosis of acute pancreatitis due to cystopancreatic duct fistula and also left-sided portal hypertension due to splenic vein thrombosis. Pathological examination reported a final diagnosis of hydatid cyst. To the best of our knowledge, coincidence of cystopancreatic duct fistula and splenic vein thrombosis due to pancreatic hydatid cyst has not previously been reported.