A 2-year-old girl developed small tense bullae on the diaper area, face and extremities. Immunofluorescent studies revealed linear deposits of IgA, IgM and C3 at the basement membrane zone (BMZ). In addition, IgA2 deposits, as well as IgA1, were clearly demonstrated in the lesional skin. However, in circulation, only IgA1 anti-BMZ antibodies were found. Immunoelectron microscopic study showed IgA deposits just underneath the basilar surface of the basal cells, which seemed to be associated with hemidesmosomes. The survey of the literature suggests that the involvement of IgA2 is extremely rare in this disease.