The first-trimester sonographic findings in a fetus with Nager syndrome are described. The most striking sonographic features included severe micrognathia, bilateral zygomatic bone hypoplasia, and abnormal low-set ears in association with upper and lower lib anomalies. The diagnosis was confirmed by clinical exome sequencing after termination of the pregnancy. This is the third case of Nager syndrome detected in the first trimester and the first to provide outstanding imaging of the facial dysmorphic features. Transvaginal three-dimensional sonography proved very useful in documenting the characteristic facial anomalies of the syndrome. Molecular genetic analysis plays an important role in confirming the diagnosis and was essential for the differential diagnosis with other acrofacial dysostoses.