Epidermolysis bullosa (EB) is a heterogeneous skin disorder that is sometimes associated with genitourinary involvement. We report our experience with the urological problems seen in these patients. Dermatology and nephro-urology databases were used to identify EB patients with urological problems, between 1980 and 2004. In total, five patients, all male, were identified, and the hospital records were reviewed. Genitourinary complications of EB present difficult challenges. The disorder can lead to urothelial blistering and strictures that can result in obstruction throughout the urinary tract. In addition, urethral instrumentation and surgical reconstruction in patients with EB has a high complication rate and should be avoided whenever possible.