Epidermolysis bullosa simplex (EBS) is an inherited blistering disease characterized by intraepidermal cleavage (Gedde-Dahl and Anton-Lamprecht, 1990;Fine et al, 1991). A very rare subset of EBS, termed "EBS superficialis" (EBSS), has been described in two families byFine et al (1989). Skin biopsy of these patients shows clefts of variable size just beneath the level of the stratum corneum, which can be completely separated from the rest of the epidermis in some cases. In two of the patients reported, there are also some clefts in the lower one-third of the epidermis. Together with this unusual clinical picture, most of the patients show atrophic scarring, milia, nail dystrophy, and blistering involving the oral cavity. After the first description of EBSS in two unrelated families, no other cases have been reported, emphasizing the rareness of these findings.