Microtia, a malformation disorder resulting from incomplete embryonic development of external ear structures, is relatively rare, is more prevalent in males, and often affects the right side in unilateral cases. It ranges in severity from minor abnormalities to complete absence of the auricle (anotia). Microtia can occur as an isolated condition or as part of syndromes like the oculo-auriculo-vertebral spectrum (OAVS) and has strong genetic associations, with common syndromes including Treacher Collins, Townes-Brock-Goldenhar, and Nager syndrome. Initial treatment typically involves non-interventional techniques and prosthetics, with surgical repair performed at older ages, using techniques such as autologous rib cartilage-based reconstruction. Conductive hearing loss is common in microtia cases, necessitating early auditory evaluation with auditory brainstem response (ABR) testing and consideration of hearing devices before the age of 4 months and surgical intervention after the age of 5. While CT scans are not recommended in early infancy, they are valuable for assessing temporal bone structure and detecting associated anomalies like congenital cholesteatoma. Renal abnormalities are frequently observed in microtia patients, suggesting the importance of routine early ultrasound examinations in these cases.