Solid pseudopapillary tumor (SPT) of the pancreas is a rare tumor of uncertain histogenesis characterized, as the name suggests, by a cystic and solid pattern of growth with formation of pseudopapillae. Accounting for only a small percentage of pancreatic neoplasms, SPT occurs primarily in young women, although cases in older patients and men have been reported. The tumor is thought to have low-grade malignant potential, as the majority of the cases are cured by simple but complete surgical resection. Knowledge of the unique morphologic and demographic characteristics of this neoplasm is essential for accurate diagnosis. Herein, we review the clinical and pathologic features, which can help separate SPTs from other primary pancreatic tumors. © 2008 Lippincott Williams & Wilkins, Inc.