Solid pseudopapillary neoplasm of the pancreas is a rare tumor of uncertain histogenesis, described separately by Gruber and Frantz, that accounts for between approximately 1% and 3% of pancreatic neoplasms. It is characterized by a cystic and solid pattern of growth patterns with formation of pseudopapillae. It occurs primarily in young women, although cases in children and older patients and men have been reported. The tumor is of low-grade malignant potential, as the majority of the cases are cured by simple but complete surgical resection. Knowledge of the unique morphologic characteristics of this neoplasm is essential for the correct diagnosis. We review herein the pathologic and immunohistochemical features of this neoplasm and its differential diagnosis with other pancreatic tumors.