This case report describes a patient with cutaneous signs in the genital and peri-anal region suspicious of Crohn’s disease without any intestinal symptom or sign. Inflammatory bowel disease can be associated with some cutaneous signs. However, in this paper, we report a patient with isolated cutaneous Crohn’s disease which is very rare (less than 100 case reports across the world). Our patient presented with inflammatory indurated plaques and sinuses in the genital region since 3 years ago. Colonoscopy showed no findings related to Crohn’s disease in the ascending, sigmoid, and descending colon, particularly the terminal ileum. The patient`s symptoms were relieved following treatment with infliximab. To the best of our knowledge, this is the first report of isolated cutaneous Crohn’s disease in Iran.