Solid pseudopapillary pancreatic tumour is an uncommon disease including 2.7% of exocrine malignancies of the pancreas. Its low incidence is associated with an uncertain prognosis and with difficult diagnostic and therapeutic problems, despite routine use of ultrasonography, TC and RMN. A case of solid pseudopapillary pancreatic tumour in a young woman is reported: the clinicopathologic features, diagnostic imaging and surgical treatment are discussed. Surgery is the primary option. Prognosis is however not fully known. From a review of the literature it is suggested that these tumours should be regarded as potentially malignant.