Pemphigus vulgaris is a severe disease of the skin and mucous membranes that is characterised by the formation of bullae. It is classified as an autoimmune disorder. The case of a very severe form of this disease in an 81-year-old woman is described. Typical lesions were present on the mucosa of her oral cavity and on the skin of the lower lip and several sites of her face. Clinical, histological and immunohistological findings confirmed the diagnosis of pemphigus vulgaris. The patient responded well to a combined immunosuppressive therapy with Prednison (Léčiva, Czech Republic) and Azamun (Wellcome Foundation, Great Britain). At 7-year follow-up she was free from complains. Her case demonstrated that, as in 70 to 90 % of the patients with this disease, the primary lesions were present on the oral mucosa many years before they manifested themselves as typical dermal signs.