BACKGROUND: Solid pseudopapillary tumour (SPT) of the pancreas is a relatively rare entity which most commonly occurs in young women. In this paper we report our clinical experience together with the current knowledge on the diagnostics, treatment and prognosis of this rare tumour. METHODS: We reviewed hospital records of patients diagnosed with a solid pseudopapillary tumour of the pancreas between January 2002 and December 2011 at the Department of Surgery, University Hospital Hradec Králové, Czech Republic. Clinical, operative, pathological data were obtained on all the patients. RESULTS: Over the 10-year period of the study we performed 181 planned pancreatic resections in our department. Overall, the 30-day postoperative mortality rate in this series of patients was 2.2%. SPT was diagnosed in 4 cases. All the patients were women and the average age was 34 years. Preoperative endosonography with biopsy sample was performed in all the patients and the diagnosis of SPT was known in all the patients before the surgical procedure. CONCLUSIONS: The current knowledge of SPT is based only on case reports and small series. It typically occurs in young women and therefore the presence of a large pancreatic mass in a young woman may suggest a diagnosis of SPT. SPT has a low malignant potential and the prognosis is excellent following complete surgical resection in the majority of the cases.