Infantile perineal protrusion, a relatively newly recognized condition, is underreported in both the dermatologic and pediatric literature. The name “infantile perineal protrusion” has evolved based on the typical anatomic location, morphologic features, and prevalence in prepubertal children. It occurs in 3 settings: constitutional (sometimes genetic or familial); functional (after constipation, diarrhea, or other irritant exposure); or associated with lichen sclerosus et atrophicus. Recognition of infantile perineal protrusion by dermatologists and pediatricians has many implications regarding proper diagnosis and management. The condition may be mistaken for condyloma acuminata or as a sign of trauma, leading to an erroneous investigation of sexual abuse. In this article, we report two new cases and make a thorough review of the literature to elucidate the mechanisms, diagnosis, classification, and management to clarify this often misdiagnosed condition.