Frantz pancreatic tumors are an infrequent pathology, histogenesis remain controversial, they occur predominantly in young women and because of their low-grade malignant potential, complete resection means good survival. The aim of this review is to present a retrospective analysis of two cases, which, because of their diagnostic difficulties can teach a lot in the evaluation process of this pathology and of its importance in the abdominal tumors differential diagnosis in children, which, when well managed with the adequate surgical method, allows the patients an excellent recovery and prognosis.