Perineal hernias (pelvic floor hernias) are extremely rare occurring through defects in musculature of the pelvic floor. This report presents a successfully treated case of primary perineal hernia and takes a review of the existing literature. The case of a 14-month-old girl with a great perineal hernia is presented. Diagnosis was secured by barium enema. The pelvic defect was successfully treated by primary suture with prolene. The literature shows many different approaches for treatment of perineal hernia, such as open or laparoscopic mesh repair, and perineal, abdominal or combined access in the adult, but our case like others confirms that primary closure of the hernial orifice through a perineal approach is also feasible in children.