Abstract: Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare pancreatic tumor with low-grade malignancy. Surgical resection is the preferred therapeutic approach for SPN patients, which has favourable prognosis with extremely low incidence of recurrence and metastasis and 5-year survival rate as 95%. With the improvement of radiological and pathological diagnosis, the detection rate of SPN is increasing. However, its pathogenesis is still unclear. Meanwhile, the diagnostic criteria and treatment strategies of SPN require standardization. Herein, the authors review the current status and advances in SPN based on related literature and clinical experience of the authors′ team on treatment of SPN, in order to deepen the knowledge of SPN, improve the ability for the preoperative diagnosis, promote standardized treatment and maximize benefits of the patients.