Cloacal malformations are a part of a large spectrum of anorectal malformations. Congenital cloaca and cloacal exstrophy represent one of the most complex malformations of the urinary, genital and gastrointestinal system. Latest research dismisses the theories of active septation of the cloaca by the descent of the urogenital septum, and provides evidence of passive division through a process called transformation. Congenital cloaca is defined as the confluence of the urethra, vagina, and rectum into a single common channel, and it occurs only in females. The diagnosis is a clinical one, made by identifying a single perineal orifice, and confirmed by endoscopy and imaging technology. The length of the common channel has to be measured, as it will determine the approach to reconstruction. The initial treatment of a neonate with a cloacal malformation includes drainage of the urinary tract / hydrocolpos, and bowel diversion by an ostomy. The definitive reconstruction of a cloacal malformation is a complex surgical procedure that needs to be done by an experienced pediatric surgical team. The most common procedure is the posterior sagittal approach with total urogenital mobilization. The cosmetic and functional result can be excellent, depending on the degree of the initial malformation. Meticulous anatomic reconstruction and comprehensive postoperative care results in as many as 50% of patients having voluntary bowel movements, urinary control, and preserved reproductive capacity. All patients must be followed on a long-term basis by a multidisciplinary team.