Ear malformations represent 50% of ear, nose and throat malformations. Ear malformations cause conductive hearing loss (CHL) and/or sensorineural hearing loss (SNHL) with a significant childhood disability worldwide. Early accurate diagnosis and treatment are mandatory to enhance language and speech development. Understanding the embryology of the ear explains the outcome of ototoxic prenatal insult according to the affected gestational age and the incidence of association among inner, middle, and external ear malformations. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations of the temporal bone are used in the evaluation of ear malformations. In this review article, the spectrum of ear malformations is discussed in detail with hints on the ear embryology, the ear radiological anatomy, and radiological determinant factors of operative reconstruction of ear anomalies.