Epidermolysis bullosa(EB), a heterogenous group of mechanobullous disease in which minimal trauma during fetal life results in blisters at skin and mucous membranes. EB is divided into three groups on the basis of the level of tissue separation within the dermal-epidermal basement membrane zone. EB simplex(EBS); intraepidermal skin separation, Junctional EB(JEB); separation in lamina lucida and Dystrophic EB(DEB); sublamina densa basement membrane zone separation. JEB is important so that rare and especially high during the first year of the life. Our case was a 21-year-old primigravida and was referred to our department of obstetrics and gynecology because of oligohydramniosis at 30 weeks of gestation. After that intrauterine fetal loss was occurred and postpartum pathologic diagnosis was JEB Genodermatoses should be kept in mind at unknown cause of intrauterine fetal loss and must be given genetic counselling at diagnosed as JEB
Keywords: Junctional epidermolysis bullosa, intrauterine fetal loss, genetic counselling