The oral cavity presents with a variety of pathological lesions ranging from benign to malignant. Autoimmune mucocutaneous disorders of the oral cavity constitute an important group of lesions that pose diagnostic, therapeutic, and prognostic difficulties to the dental clinician. Many of the lesions may become potentially fatal if they are undiagnosed or misdiagnosed in their early stages. Pemphigus vulgaris is a potentially life-threatening autoimmune mucocutaneous disorder that causes blisters and erosions of the skin and the mucous membrane. It manifests clinically as vesicles, bullae, or desquamative gingivitis and histopathologically shows the presence of acantholysis. 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 lesions are critical as they may prevent skin involvement. The incidence of pemphigus vulgaris occurs in a ratio of 2:1 (female: male). Adequate knowledge of diagnostic and therapeutic modalities helps the clinician in appropriate management. In this article, we report a case of oral pemphigus vulgaris in a 34-year-old male.