For the first few years of life this 4-year-old girl experienced diffuse evere recurrent blistering of the skin and mouth especially on the face, neck, and distal extremities. As a consequence she developed dental caries, nail dystrophy, keratoderma of the palms and soles, and subtle skin atrophy especially on the face and extremities. Although her skin remained fragile, she only rarely developed new blisters particularly during the summer. Severe gastroesophageal reflux resulting in poor feeding, failure to thrive, and recurrent aspiration was managed with antacids and gastrostomy placement. The diagnosis was confirmed with gene markers in the patient. Neither parent carried the mutation.