Severe aplasia of the ear raises both a cosmetic and a functional problem. Surgery is often performed starting at four or five years of age but early management is essential. The need for a hearing aid should be evaluated at the age of six months. At birth, the infant should have investigations for concomitant malformations, which are common, and for etiologic factors. Functional surgery to create a canal, tympanic membrane, and chain of ossicles should be performed only in bilateral forms. Satisfactory cosmetic results can be achieved by cartilage autografting according to Brent's technique.