Perineal groove is a rare perineal congenital malformation that usually affects newborn females. We are presenting two cases of this rare congenital anomaly who underwent a conservative approach. In this article, we are highlighting the clinical course for the expectant management of the perineal groove as well as reviewing the literature about the perineal groove to improve the recognition and the diagnosis of this uncommon malformation. To the best of our knowledge, we could not find any case of a perineal groove associated with midline craniofacial anomalies (bifid nose) which was one of our cases.
