AIM: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm, its preoperative diagnosis is difficult and therefore inappropriate therapy or postoperative complications are frequent. Reviewing the literature, the purpose of this article was to identify guidelines to improve diagnosis and treatment of SPT. CASE REPORT: Authors report a case of SPT of the pancreas in a 27 year-old woman in whom a mistaken radiologic diagnosis made surgical strategy difficult and caused postoperative complications. DISCUSSION/CONCLUSIONS: Clinicians and surgeons should: 1) consider the possible disease of SPT in young females, with pancreatic encapsulated lesion with well-defined borders and variable central areas of cystic degeneration, necrosis or hemorrhage showed on radiological examinations. 2) Intensify the differentiation of the clinical symptoms, especially during the course of therapy of chronic gastritis and diabetes. 3) Use immunohistochemical stains of alpha-1-antitrypsin, alpha-1-antichymotrypsin, vimentin and neuron-specific enolase. 4) Keep this unusual but potentially curable tumor in mind, following patients who had suffered from acute pancreatitis or abdominal injury. Increasing experience with this tumor leads to a greater awareness of its clinical presentation and pathological features and a lower rate of misdiagnosis. 5) Finally, perform, where technically feasible, conservative surgical treatment, that is safe and effective.