Pseudopapillary tumor of the pancreas is a rare exocrine tumor of the pancreas. It was first reported by Frantz in 1959 and was officially named as pancreatic solid pseudopapillary tumor by WHO in 1996, which often occurs in young women with an average age of about 25 years. Most patients are found accidentally during physical examination, while a few patients present with abdominal pain or abdominal masses. The tumor can occur in any part of the pancreas, but the caudal part of the pancreatic body is more common. Recent advances in impact medicine have significantly improved the rate of preoperative correct diagnosis of pancreatic solid pseudopapillary tumors, and enhanced CT and MRI have good diagnostic value. Since pancreatic pseudopapillary tumors are tumors with a low malignant tendency, they should be aggressively surgically removed once detected. The development of minimally invasive surgery has eliminated the need for open surgery for most patients with tumors located in the tail of the pancreatic body, and in addition, the spleen is no longer routinely removed. As long as the short gastric vessels are preserved, most spleens can be preserved without complications such as infarction and infection. After complete resection of solid pseudopapillary tumors of the pancreas, follow-up treatment is usually no longer required and the majority have a good prognosis.