Pemphigus vulgaris is a rin, chronic intraepidermal bullous disease with potentially fatal outcome. Oral lesions precede skin lesions in at least $70\%$ of cases, and in cutaneous disease, concomitant oral lesions are encountered in $90\%$ of patients. This disorder involve the skin and mucous membranes, especially the oral and pharyngeal mucosa, but may also involve the nasal, oropharyngeal, laryngeal and esophageal mucosa. Oral lesions are initially vesicobullous but rapidly rupture, leaving a painful erosion that shows little tendency to heal. Pemphigus vulgaris affecting the oral mucosa is still diagnosed only after considerable delay, because oral ulceration in common, and clinicians believed the lesions to be caused by more common conditions such as recurrent aphthous stomatitis rather than a rare disorder such as pemphigus vulgaris. The definitive diagnosis of pemphigus vulgaris should be undertaken as early as possible, so that treatment can be started at an earl·y stage. Because of the presence of nonspecific oral ulcer, high degree of suspicion is often required to ultimately make the diagnosis of pemphigus vulgaris and then we report a case of pemphigus vulgaris with a literature review.