The management of a unilateral congenital atresia of the external auditory canal is nonuniform and debated. Various surgical approaches, timing, coordination with microtia repair, and variable hearing improvements all contribute to the debate regarding management of this entity. This paper outlines our craniofacial team approach to the congenital unilateral atresia and microtia in children. Selection criteria, timing of repair, coordination with microtia repair, surgical results, and pitfalls will be discussed. The results of surgery in 16 patients with unilateral congenital atresia of the external auditory canal and 2 children with bilateral atresia will be presented. Repair of the atresia was undertaken in children 5 years or older who had pneumatized mastoids and middle ears. Replacement of the malleus/incus complex with a partial ossicular reconstruction prosthesis improved closure of the air-bone gap. Drawbacks included meatal stenosis and deepithelization of the split thickness skin graft lining the external auditory canal. Repair of the unilateral congenital atresia is a demanding and challenging problem but one in which excellent results are achievable.