The risk of malignant change in a congenital nevocellular nevus is approximately 10 percent and is the chief indication for early excision. In 90 percent of patients, removal is simple; the remainder present with major cosmetic deformities and are difficult to manage successfully. Seventeen children (newborn to 17 years of age) were treated, including 11 children with localized lesions and 6 children who presented with abnormalities that covered 5 to 50 percent of the total body surface area. In those cases not amenable to primary removal, serial excision was performed at an average of 6 month intervals. Staging was determined by the softening and mobility of the surrounding tissue at follow-up evaluation. No effort was made to provide a cosmetic closure until the final stage. Utilizing this technique, complete removal of congenital nevocellular nevi is possible without the need for disfiguring skin grafts, even for the giant variety. Early excision obviates the risk of malignancy and provides satisfactory cosmetic results.