Congenital nevi are a group of skin lesions occurring at birth or becoming apparent within the first several years of life, and are characterized by ectopic rests of dermal elements. Although most commonly melanocytic in nature, nevi may also originate from sebaceous, neural, or epidermal elements. Lesion characteristics vary according to the type of cell involved, location within the skin, and level of cell differentiation. Knowledge of the differential diagnosis and natural history of these lesions can help balance the plan of care, in order to address the potential risk of malignant degeneration while accounting for functional and aesthetic concerns encountered in the course of excision and reconstruction. This chapter outlines the presentation and management of congenital nevi, with special emphasis on congenital melanocytic nevi (CMNs).