Nevus lipomatosus cutaneous superficialis resembling rudimentary male external genitalia in a neonate

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare developmental abnormality characterized by group of ectopic fat cells dispersed in collagen bundles of the papillary dermis. Herein we present a case of congeniatal solitary NLCS in the perianal region of a female neonate presented with features of pseudo hermaphrodism which was ruled out by routine investigation. Simple surgical exicision was done and histological examination was suggestive of NLCS.