Oral mucous membrane pemphigoid (OMMP) is a rare inflammatory autoimmune mucocutaneous disorder. Oral mucosa is affected in 90% of cases. Its development is chronic, with a possible involvement of ocular, laryngeal and genital mucosa. Spontaneous remission is rare. Although the etiology of OMMP is still unclear, there is evidence that it is a complex autoimmune disease mediated by autoantibodies directed against different components of the basement membrane zone. Cellular immunity is also involved in the pathogenesis. Atrophic and erosive lesions are the sequelae of the rupture of the subepithelial blisters. The treatment is usually aimed at relieving pain and may include corticosteroids and immunosuppressive agents. However, the use of these drugs may be inefficacious. Oral hygiene and follow-up are mandatory for patients affected by OMMP. It is important to increase awareness of oral lesions in autoimmune blistering/erosive dermatologic conditions.