Diseases affecting the inner ear can be broadly divided into congenital malformations and acquired pathologies. Inner ear malformations (IEMs) result from arrested development of inner ear structures during the first and second trimesters of fetal life. Sensorineural hearing loss is the primary clinical manifestation and is usually detected in the newborn period through screening programs. From an imaging perspective, about 80% of IEMs show no detectable abnormality on CT or MRI due to late developmental arrest affecting the membranous labyrinth. The remaining 20% show bony labyrinth abnormalities that are readily identified on imaging. Imaging plays a crucial role in treatment planning, including suitability for cochlear or auditory brainstem implantation. Major malformations include Michel deformity, rudimentary otocyst, cochlear aplasia, common cavity, and cochlear hypoplasia. Incomplete partition anomalies (IP-1, IP-2/Mondini, and IP-3) involve abnormal internal cochlear architecture with normal external size. Other important entities include enlarged vestibular aqueduct, cochlear nerve deficiency, and isolated semicircular canal dysplasia. MRI is essential for evaluating inner ear tumors, labyrinthine hemorrhage, and endolymphatic hydrops in Ménière’s disease.