Perineal epidermoid lesions are uncommon, with only a few research papers accessible on the subject. Because these lesions are varied in origin and can range from benign to malignant, it can be difficult to tell them apart. A wide variety of lesions are evaluated in the differential diagnosis of perineal cystic lesions, however perineal epidermoid cyst is uncommon. An epidermoid cyst is a benign ectodermal congenital abnormality. Epidermoid cysts can be found all over the body, however, they are uncommon in the perineal area. We discuss a case of a perineal epidermoid cyst in an adult female presenting to the general surgery department with painful perineal swelling. The patient was advised for an ultrasound (US) and MRI for further evaluation and the findings are reported.