Abstract A 40‑year‑old man presented with recurrent perineal abscesses originating from a congenital isolated perineal canal extending from the anterior aspect of the anal canal to the base of the scrotum. Since the age of 18, he experienced 3‑4 episodes of painful perineal swelling requiring incision and drainage. The chronicity and midline perineal involvement raised repeated concerns for underlying Crohn’s disease. However, serial MRI pelvis studies, colonoscopic evaluations, and histology from the mucosa‑lined tract consistently showed no evidence of inflammatory bowel disease. This case highlights a rare congenital anomaly that can closely simulate perianal Crohn’s disease, leading to diagnostic uncertainty and repeated interventions over decades.