We present a case where a baby girl was born with failure of fusion of the perineal raphe. To our knowledge, there has been limited reports of this form of congenital abnormality in the literature and is referred to as "perineal groove." The incidence of perineal groove is unclear but it is considered to be rare. This case reviews the embryological origin of this congenital abnormality.
(c) 2009 Wiley-Liss, Inc.