Solid pseudopapillary neoplasm of the pancreas, solid and cystic, is a rare disease compared to ductal adenocarcinoma. The tumor most often affects women of African race aged in their twenties or thirties. We report the case of a 48-year-old man affected by solid pseudopapillary neoplasm of the pancreas treated by distal splenopancreasectomy. The patient was discharged on the 10th postoperative day in good general condition, feeling normal and with blood chemistry values within normal limits. The main characteristic differentiating papillary tumors of the pancreas from ductal adenocarcinoma is that in the latter case surgical eradication is a definitive solution and no other treatment is required, as confirmed by our case and those reported in the literature.