Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare neoplasm which primarily affects young women without specific clinical manifestation. Computed tomography and magnetic resonance imaging contribute to the preoperative diagnosis of SPN. Surgery is the main treatment approach for SPN and more research of radiation and chemotherapy is needed. Tumor diameter larger than 5 cm, tumor infiltrating nerve, vessel or adipose tissue can increase the risk of recurrence. However, these patients can still get good prognosis after surgical resection.