The aim of this study is to describe external, middle and inner ear malformations in children with congenital craniofacial abnormalities. Seventeen patients with craniofacial and external ear anomalies, aged between 12 days and 15 years (mean 3.4 years), were studied. The majority of children had conductive hearing loss. High-resolution CT of the petrous bone was performed in all cases; in 9 cases three-dimensional reconstructions were done. For each child CT findings of external, middle, inner ear, facial nerve and skull base were analysed. Ear malformations have been correlated with anomalies of the auricle. The most serious auricular anomalies were more frequently associated with external auditory canal atresia, dysplasia of the tympanic cavity and malleo-incudal joint and aberration of the mastoid segment of the facial nerve. These anomalies are probably related through a common embryological origin. Inner ear malformations were diagnosed more rarely, but we emphasize that these malformations are more frequent in “syndromic” forms with craniofacial abnormalities rather than in isolated anomalies of the auricle; it is very important because inner ear abnormalities are considered a contraindication to functional surgery in patients with atresia. Diagnostic imaging is useful for an early and complete characterization of the craniofacial and ear malformations; a precise analysis of the ear abnormalities is necessary to give an indication for the eventual audiological treatment. High-resolution CT is the method of choice for the analysis of the external and middle ear; CT and MR should be integrated for the study of the inner ear and facial nerve. CT and MR are helpful to determine the extent of craniofacial abnormalities; MR should be done when encephalic malformations are suspected. Finally, three-dimensional CT is useful for the maxillofacial surgeon to plan surgical treatment.