Objectives The ear’s congenital anomalies usually occur in the outer, middle or inner ear separately or in combination. We had determined the сongenital ear’s anomalies (CEA) in newborns (2006-2018 yy) in West Ukraine, Lviv region. Methods 14 newborns who were diagnosed according to the reporting form by the maternity hospitals with CEA were analyzed over a period of 13 years.Results During the study period (2006–2018), there were 366 147 births reported, and 8634 newborns with birth defects, 14 were with CEA, an average incidence of 0.4 per 10 000 births. Our study included 10 boys and 4 girls with CEA. Male-to-female ratio was 2.5:1. The number of newborns with ear pathology was 1.8 times higher in the city than in the village. The most common birth defects of the ear were Q16.1 congenital absence, atresia and stricture of auditory canal (external) – 4 (28.6%) and Q16.9 CEA causing impairment of hearing, unspecified – 4 (28.6%), especially the most common of right side. Microtia (Q17.2) was met very rarely -1 (7.1%) and other CEA Q16.9-1 (7.1%). During this period there were only 2 cases of anotia and 1 case of microtia. There was only 1 (7.1%) case of bilateral CEA in boy from town. Just one girl had CEA was associated with birth defect of musculoskeletal system. Conclusion This is necessary to establish the correct diagnosis in time, especially for mind of the family doctors, genetic counseling for relatives family for has benefited earlier prophylaxis.