Genetic alterations whether germline or somatic in any of the skin layers, cells, and ancillary parts can create nevoid processes within the skin. These developmental changes and nevi often present in the first 2 years of life. Sometimes, exogenous stimuli, including puberty, promote new or altered lesional growth. Some of these alterations which are clinically apparent are maturational and may not be nevoid or mosaic in nature. In this chapter, congenital or early-onset developmental lesions are discussed.