Iran is an endemic region for hydatid disease. Primary pancreatic hydatosis is a rare event; severe acute pancreatitis related to pancreatic hydatid cyst is extremely rare.We present the case of a patient with severe acute pancreatitis and hydatosis of the pancreas without liver or lung involvement. All etiologies for acute pancreatitis were excluded. The patient underwent a distal pancreatectomy which led to the diagnosis of pancreatic hydatosis.Since hydatid disease is endemic in some regions, it should be considered as one of the underlying etiologies for inflammation or infection of the pancreas (acute pancreatitis).