The management of a child with congenital ear malformation, in particular if the external ear is severely involved, is difficult because of the complexity of the therapeutic problem, and that of parental anxiety. It is very important to plan a complete therapeutic/habilitative programme as soon as possible, even if surgical procedures are delayed. Diagnostic imaging plays an important role in the global assessment of a child with microtia, in order to diagnose possible associated external auditory canal, middle and inner ear malformations. For these reasons our diagnostic protocol for children with microtia includes otological and audiological evaluation, clinical genetics and radiological imaging, from the neonatal period. Here, data are reported on 27 children with microtia who completed the diagnostic protocol. In eight of 27 cases microtia was bilateral: in unilateral cases the right side was affected more frequently. Other congenital malformations were diagnosed in 41% of cases. A high correlation between the degree of microtia and the frequency of external and middle ear dysplasias was found, in accordance with larger studies of the literature. Inner ear malformations were found less frequently, but without apparent correlation with the degree of microtia. The fact that children with microtia may also have severe inner ear malformations is emphasized.