Objective: To summarize the experience in diagnosis and treatment of solid pseudopapillary tumor of the pancreas (SPT). Methods: The clinical data of 42 patients with SPT admitted between June 2000 and May 2011 were retrospectively analyzed. Results: Of the 42 patients, 41 (98%) cases were female and only one case was male, and their ages ranged from 16 to 63 (average of 31.1). In nine patients the tumors were located in the head of the pancreas, three were located in the neck and 29 were located in the body and tail. The maximum tumor diameter varied from 2 to15 cm (average of 6.3 cm). Twenty patients had no obvious signs on admission, 21 patients had only vague upper abdominal pain or discomfort, and only one case presented with severe abdominal pain and high fever due to tumor rupture. Lesions in the pancreas appeared as hypoechoic, isoechoic, or complex masses (having both cystic and solid components) at ultrasound, and presented as roundish cystic/solid masses on CT scan where the solid component showed different degrees of enhancement after contrast-enhanced imaging. All patients underwent surgical resection and the lesions were confirmed as SPT by intra- and postoperative pathology. Of the patients, two cases underwent pylorus-preserving pancreaticoduodenectomy, two cases had duodenum-preserving pancreatic head resection, spleen-preserving pancreatic body and tail resection was performed in 10 cases, pancreatic body and tail resection in 13 cases, local resection in 14 cases, and one case received palliative resection. Forty patients were followed up for 6 months to 11 years (average of 49 months); all of them were alive and with no distant metastases, and one of them who experienced local recurrence 8 years after local resection underwent a second local resection and survived to date for 41 months. Conclusion: High vigilance should be maintained for SPT in young women with the complex cystic/solid pancreatic masses. Due to low-grade malignancy, patients can obtain long-term survival through complete surgical resection and with organ function preservation as far as possible.