Epidermoid cyst is a common, cutaneous, benign lesion that is frequently seen on the face, neck and trunk. It is rarely seen in the perineal region. The differential diagnosis of perineal cystic lesions includes a wide range of lesions, with an epidermoid cyst being a rare entity to be considered, and only a few such cases have been reported in the literature. Our case was a young Asian female who presented with painless perineal swelling for the past 6 months and was diagnosed as having an epidermoid cyst on the basis of diffusion-weighted MRI and contrast enhancement pattern, which was later confirmed by histopathological findings.