A case of epidermolysis bullosa letalis in a newborn is described by the authors. The girl had numerous lesions for all the tegument except on the face, palms and soles. Something of them were bullous. The histopathological examination revealed a bulla with detachement dermoepidermic. Without infiltrate in dermis. The roof of the bullae is conservate. In the PAS stain we show the detachement over of the basal membrane.