BACKGROUND Pemphigus is a potentially life threatening autoimmune disease that causes blisters and erosions of the skin and the mucous membrane. The epithelial lesions are a result of auto-antibodies that react with desmosomal glycoproteins that are present on the cell surface of the keratinocyte. The autoimmune reaction against these glycoproteins causes a loss of cell to cell adhesion, resulting in the formation of intraepithelial bullae. Eighty to ninety percent of patients with pemphigus vulgaris develop oral lesions and in 60% of cases oral lesions are the first sign. Timely recognition and therapy of oral lesion is critical as it may prevent skin involvement. If treatment is instituted during this time, the disease is easier to control and the chance for an early remission of the disorder is enhanced. METHODS This case report describes the case of a patient who complained of ulcers of the mouth and difficulty in swallowing since 20 days, who was diagnosed as having Pemphigus vulgaris. Due to early diagnosis, lower doses of medication for a shorter period of time could control the disease. CONCLUSIONS Dental professionals must be sufficiently familiar with the clinical manifestations of pemphigus vulgaris to ensure early diagnosis and treatment which in turn determines the prognosis and course of the disease.