Dermatitis herpetiformis and bullous pemphigoid are bullous autoimmune diseases of the skin microscopically characterized by subepidermal blisters. We present a 77-year-old patient with an 18-month history of disseminated pruritic papular lesions. Direct immunofluorescence microscopy revealed linear deposition of IgG at the basement membrane zone as well as granular deposits of IgA in the papillary dermis. Circulating IgG antibodies against BP180, BP230 and gliadin as well as IgA reactivity against endomysium, tissue transglutaminase, and gliadin were detected compatible with both bullous pemphigoid and dermatitis herpetiformis. Here, we review the English literature on all previously reported patients with co-occurrence of both entities. Interestingly, in previous cases, tissue-bound and serum autoantibodies against the respective target antigens had not yet been completely characterized.