Involvement of the genitourinary tract has been described in many different types of epidermolysis bullosa (EB). Pathology may be broadly divided into problems resulting in obstruction, that may in turn lead to hydroureter or hydronephrosis, or disease primarily affecting the renal parenchyma. Left unrecognized and untreated, renal tract disease may lead to chronic renal failure, and consequent problems associated with providing renal replacement therapy. Management of the urogenital tract in EB should therefore focus on detecting symptoms suggestive of obstruction and regular monitoring to detect problems as early as possible.