Immunoglobulin A (IgA) pemphigus is a clinically distinct variant of pemphigus characterized by intercellular IgA deposition in the epidermis. Recently, an IgA/Immunoglobulin G (IgG) subset of pemphigus with IgA and IgG anti-keratinocyte cell surface antibodies has been described. Both IgA and IgA/IgG pemphigus have been associated with internal malignancies. Above all, monoclonal IgA gammopathy in patients with IgA pemphigus has been reported, and lung cancers in association with IgA/IgG pemphigus have been described. IgA pemphigus can be successfully treated with dapsone, whereas therapeutic management of IgG/IgA pemphigus is not well established yet. We report a rare case of a patient, who developed atypical pemphigus with both IgA and IgG autoantibodies and an underlying lung cancer, successfully treated with corticosteroids and dapsone.