Perineal angiomyxoma is a rare entity, more commonly seen in females. We report a case of a 44 year old male who presented with a perineal swelling which became prominent with penile erections. Magnetic Resonance Imaging (MRI) revealed a T1 hypointense and T2 hyperintense midline lesion (4.6 × 2.5 × 5 cm) in relation to corpus spongiosum, with ill defined fat planes with the bulbospongiosus muscle and progressive enhancement on dynamic contrast sequence. A differential diagnosis of soft tissue sarcoma or hemangioma was made and the mass was completely excised via a midline perineal incision. The histopathology revealed features consistent with angiomyxoma.