Cloacal exstrophy is an extremely rare congenital disorder first described by Littre in 1709.1 It is the most severe form of the bladder exstrophy–epispadias complex and is characterized by findings of two exstrophy hemibladders, exstrophy hindgut, underdevelopment of hindgut, pubic diastasis, prolapsing terminal ileum, complete separation of genitalia, and omphalocele (Figure 81.1). The first successful treatment of cloacal exstrophy using a three-stage procedure was described by Rickman in 1960.2 Since then, advances in medical and surgical care have improved survival to over 80%.3 , 4 With this drastic increase in survival, the goals of care have shifted toward improvement of early diagnosis, preservation of the function of affected organs, and improvement in quality of life.