Solid pseudopapillary tumor of the pancreas (Frantz's tumor) is a very rare disease with uncertain malignant potential. This tumor occurs in young women at the age before 35 years. Asymptomatic disease course leads to growth of tumor up to the huge size. Surgery is the only treatment method applied. Histological diagnostic is complicated with similarity of tumor with neuroendocrine tumor of the pancreas. Disease is characterized with favorable prognosis. Paper shows our own clinical observation of Frantz’s tumor. During control ultrasound examination of woman aged 34 years, echo-signs of mass lesion in extraperitoneal area near the lower pole of the left kidney were diagnosed. During physical examination in the left hypochondrium a soft, atropic mass lesion 5.5x5 cm in size was diagnosed. Lesion presence is confirmed by CT assessment. Distal resection of the pancreas and splenectomy was performed. Diagnosis was verified histologically and immunohistochemically. Postoperative period is complications free. The results of CT assessment, detailed description of gross specimen and the results of histological and immunohistochemical examination are presented.