Solid pseudopapillary tumor of the pancreas is a rare neoplasm of the exocrine pancreas that predominantly affects young females. Patients may be asymptomatic or present with non-specific symptoms. Asymptomatic patients are diagnosed with an incidental finding on imaging obtained for other reasons. This entity comprises only 0.3%–2.7% of pancreatic cancers; therefore, diagnosis requires a high level of suspicion. Solid pseudopapillary tumor of the pancreas consists of a well-circumscribed, heterogeneous mass that contains solid and cystic components with some foci of calcification, and is usually located in the distal pancreas. Diagnosis may be confirmed with preoperative biopsy. Complete surgical resection is the standard of care and carries an excellent prognosis, even in the setting of large tumors or advanced disease.