Newborns can present with a host of varying birthmarks. Such nevi can represent a benign condition or a harbinger of a more concerning underlying condition or genetic syndrome. It is important for caretakers to be able to distinguish between the many seemingly similar or unusual entities. This article highlights distinguishing features of the most commonly encountered nevi seen at birth or in early childhood. Furthermore, updated information from the literature about controversies and current understanding of the genetic basis surrounding various nevi is presented.