Pemphigus vulgaris is an autoimmune bullous disease which is characterized by erosion and bullae on the skin and mucosae. The disease is thought to occur under the influence of predisposing factors in people with genetic susceptibility. Pemphigus may coexist with other autoimmune diseases such as myasthenia gravis, lupus erythematosus, rheumatoid arthritis, and pernicious anemia as well as with neoplasias such as thymoma, carcinoma, and lymphoproliferative diseases. Crohn’s disease is a chronic inflammatory disease which involves the whole gastrointestinal mucosa from the mouth to the anus. Etiopathogenesis of this disease includes autoimmunity. A 39-year-old Turkish man presented to our polyclinic due to the erythema on the trunk and the shoulders. The patient stated that his complaints had started a month before and were accompanied by pruritus. He had had a history of Crohn’s disease for 10 years. Histopathologic examination of the blister revealed pemphigus vulgaris. Physicians should be able to recognize Crohn’s disease might accompany with autoimmune skin disease. For our knowledge this is the first case with coexistence of two diseases.