Epidermolysis bullosa is an uncommon disease characterized by the formation of blisters following minor trauma. The three major types are simplex, junctional, and dystrophic. Generalized enamel hypoplasia is accepted as a feature of the junctional type. Teeth with enamel hypoplasia are liable to destruction in the form of caries and/or gingivitis. In this report, a patient with epidermolysis bullosa simplex and severe enamel hypoplasia is described and the importance of early dental consultation for all children with this disease is stressed.