Epidermolysis bullosa (EB), a hereditary, blistering form of dermatosis, can be divided into the following three main groups: epidermolysis bullosa simplex (EBS), junctional epidermolysis bullosa (JEB) and dystrophic epidermolysis bullosa (DEB). The previously known form of generalized atrophic benign epidermolysis bullosa (GABEB) is classified as junctional epidermolysis bullosa non-Herlitz (JEB-nH) today. An 11-year-old boy with junctional epidermolysis bullosa non-Herlitz with urethal involvement had a complicated course with bladder obstruction and secondary renal insufficiency. According to currently available literature, this is the earliest case known of JEB-nH with involvement of the urinary tract in childhood. Taking into account the complicated course in this young patient, it must be emphasized that in cases of epidermolysis bullosa hereditaria, uncommon affections occurring in infancy should be given careful attention, as such types of associated manifestations are mainly responsible for the lethality in this disease.