BACKGROUND: Congenital pigmented nevi of the auricle are uncommon. The authors' approach is to excise these nevi and perform reconstruction because of the risk of malignant transformation and the aesthetic and psychological effects these nevi can have on the child. This study presents the authors' experience in treating congenital nevi of the ear and suggests treatment principles and guidelines for the reconstructive surgeon. METHODS: Fourteen patients with congenital nevus of the ear were treated from October of 1992 to September of 2008 by the senior surgeon (B.S.B.). Nevi involving the more stable areas such as the concha can be resected and grafted early; the antihelix, scapha, and triangular fossa area can be resected and grafted next; and the helical rim, having the most easily distorted cartilage, should be treated last. Lobule reconstruction requires combined flaps and a dermal fat graft or a postauricular fascial fat flap. RESULTS: Successful reconstruction was achieved in 10 patients. Three patients require final revision procedures (lobule reconstruction). One patient, early in our series, developed a deformed helical rim resulting from skin grafting at age 16 months, before the cartilage was firm enough to withstand the contraction forces of the skin graft. All subsequent patients with helical rim involvement had treatment delayed until the ear was at or near completion of its growth. CONCLUSIONS: Congenital nevi of the ear present a challenging reconstruction surgeon. The authors developed a treatment plan that breaks the ear down to aesthetic units and considers the location of the nevus, patient age, and the firmness of the cartilage.