The differential diagnosis of cystic neoformations in the pancreas is challenging. We report a case of a true solitary cyst of the pancreas in a 26-year old woman. Abdominal magnetic resonance imaging and computed tomography showed a unilocular neoformation in the head of the pancreas, without obstruction of Wirsung's duct. We excised the cyst and performed Roux-en-Y loop pancreaticojejunostomy, but the patient suffered recurrent acute pancreatitis from Wirsung's duct stenosis. Thus, a new Roux-en-Y loop pancreaticojejunostomy was successfully done 6 months later. Histologically, the cyst was lined by cuboidal epithelium, immunohistochemically positive to anti-carbohydrate antigen 19-9 antibodies. To our knowledge, only 11 cases of solitary true cyst of the pancreas in adults have been reported, so the characteristics of this unusual entity are not well known. We propose a scheme for the differential diagnosis of cystic neoformations of the pancreas, starting from the histopathological definition of a true solitary cyst.