Infantile perianal pyramidal protrusion (IPPP) is an uncommon and underreported benign cutaneous lesion characterized by a protrusion from the anal orifice. It is also believed to be often mistaken for other conditions. The unawareness of this lesion may be responsible for underreporting and an excessive concern both in providers and in parents. Timely diagnosis and reassurance need to be emphasized in the provider community. We report an interesting case of IPPP on the first day of life, which was erroneously diagnosed as imperforate anus at her delivery.