Abstract: A case of pemphigoid nodularis is reported and relative literature is reviewed. A 62-year-old female presented with papules and nodules all over the body with itching for 10 years. Physical examination showed dark red papules, nodules and plaques on the trunk and limbs and no visible blister was seen. The level of IgE in blood was increased. Histopathological examination showed hyperkeratosis or parakeratosis of epidermis, acanthosis, perivascular mononuclear cells infiltration in the superficial dermis. Direct immunofluorescence and indirect immunofluorescence were negative. Anti-BP180 IgG titres were above 200 U/mL. The diagnosis of pemphigoid nodularis was made. Prednison and cyclophosphamide were given, and the patient is under follow-up.