A solid pseudopapillary neoplasm (SPN) is an extremely rare tumour of the pancreas that frequently occurs in young females and is mostly benign. SPN is a low-grade malignant tumour that may evolve years before symptoms start. However, the pathogenesis of this tumour remains unclear and there are no adequate reports of long-term results to evaluate the management and the long-term surgical control. We describe a new case of SPN with a 10-year follow-up, and review the world literature that accounts for approximately 322 cases. Moreover, a review of the current management and surgical tendencies in the treatment of SPN is considered. An SPN pancreatic tumour occurred in a 24-year-old female who complained of episodic mild abdominal pain sustained by a palpable epigastric mass. The tumour mass was detected by ultrasound and computer tomography and was localised at the tail of the pancreas adherent to the spleen. The preoperative diagnosis was uncertain and en-block distal pancreatectomy and splenectomy were performed. The size of the mass which weighed 300 g was 11 × 12 × 8 cm, and the tumour was strictly adherent and invaded the splenic hilum. Histologic examination confirmed a complete resection of the primary SPN that locally invaded spleen. The postoperative period was uneventful and after a 10-year follow-up the patient is free of symptoms. SPN should be considered in the differential diagnosis of large pancreatic masses, especially in young females. Radical resection, where technically feasible, should be considered the therapy of choice as it is a safe and effective control of the disease.