Objective To summarize the current diagnostic and therapeutic advancement of solid pseudopapillary tumor of the pancreas. Methods Relevant literatures about the diagnosis and treatment of solid pseudopapillary tumor of the pancreas, which were published recently domestic and abroad were collected and reviewed. Results Solid pseudopapillary tumor of the pancreas is now considered to be a low-grade malignancy with characteristic clinical, imaging and pathological features. Resection is considered to be the optimal choice with favourable prognosis. Conclusion An awareness of the features may guide us to a correct diagnosis and treatment of this rare neoplasm, but the deep understanding of the disease needs the accumulation of more cases and fundamental research.