Pemphigus vulgaris (PV) is a rare autoimmune bullous disease of the skin that almost always produces mucosal manifestations. PV should be considered in differential diagnosis whenever there is a history of multiple, chronic, nonhealing erosions that begin as blisters. Direct immunofluorescence of perilesional skin/mucosa remains the most valuable diagnostic procedure. The fact that blisters or more often erosions on the mucosa of upper respiratory and digestive tracts are frequently the first manifestation of the disease implies that ENT physicians have to familiarize themselves with the clinical manifestations of PV to ensure early diagnosis and proper treatment.