Restorative proctocolectomy and ileal pouch-anal anastomosis (IPAA) is considered the therapy of choice for the prophylactic treatment of FAP in adults, while straight ileoanal endorectal pull-throughs were often favored in children. However, our experience with five children undergoing an ileoanal J-pouch procedure under the age of 15 years (7-15) due to early onset of a severe symptomatic FAP phenotype suggests results which are superior to those after direct ileoanal anastomosis. Even after a primary straight ileoanal pull-through with local complications and a high defecation rate, secondary IPAA should be considered.