Objective To report a case of solid pseudopapillary tumor of the pancreas and review literatures about this disease, so as to improve clinical understanding of this rare disease and reduce misdiagnosis and missed diagnosis. Methods The clinical manifestation,diagnosis and treatment of the patient with solid pseudopapillary tumor of the pancreas were analyzed retrospectively. The clinical manifestation and key points of diagnosis were summarized from literature review. Results Literature review showed that: solid pseudopapillary tumor of the pancreas is a rare low-grade malignancy tumors, which frequently occurs in young women. This case is a thirty-eight years old female patient who was admitted to the hospital due to abdominal pain. Postoperative pathology confirmed a solid pseudopapillary tumor of the pancreas. Follow-up of 1 year showed no obvious signs of recurrence and metastasis. Conclusion Solid pseudopapillary tumor of the pancreas has no specific clinical manifestations, the diagnosis mainly depends on imaging examination and histopathological results. Surgical resection is the preferred treatment for solid pseudopapillary tumors of the pancreas.