A male with a severe form of congenital nephrogenic diabetes insipidus (diuresis 10 l per day) had megaureters, megavesica, large residuum and a history of three traumatic ruptures of the megavesica and a recurrent urinary tract infection (RUTI). Hemodialysis was introduced at 41 years of age. At age of 42, he underwent a bilateral retroperitoneoscopic nephrectomy to prevent further RUTI and 8 months later transplantation of a cadaver kidney. Intermittent catheterization is necessary due to residual urine in the urinary bladder.