We report a 71-year-old woman with antiepiligrin cicatricial pemphigoid associated with nephrotic syndrome. She presented with a six-month history of pruritic blisters over her trunk and legs. She also had episodes of recurrent painful oral erosions. A skin biopsy showed a subepidermal bulla, and a direct immunofluorescence (DIF) study revealed linear deposition of IgG and C3 at the basement membrane zone (BMZ). Indirect immunofluorescence (IIF) staining of 1 M NaCl-split skin demonstrated circulating IgG autoantibodies reactive with the dermal side. Immunoprecipitation studies of the patient's serum disclosed IgG autoantibodies directed against a set of polypeptides that corresponded to laminin 5 (beta 3 gamma 2). Based upon the long-standing edema of her legs and her hypoproteinemia, she was diagnosed with nephrotic syndrome. To our knowledge, the association of antiepiligrin cicatricial pemphigoid with nephrotic syndrome has not been reported previously.