We reported a case of papillary and cystic tumor (Frantz's Tumor) associated with familial adenomatous polyposis in a young man, 29 years old. This case emphasized the interest of fine needle biopsy and the difficulties for determination of the final pathology. Close long term follow up is essential as local recurrencies and metastasis in the long term had been recorded, undergoing resection with curative intent. Papillary and cystic tumors should be resected.