The technical improvement in image resolution of modern ultrasound equipment has led to the characterization of an increasing number of fetal facial dysmorphic features, many of which are associated with well-recognized genetic syndromes. Three cases of Brachmann-de Lange syndrome in which two- and three-dimensional second-trimester sonography depicted characteristic facial features of this condition, i.e., long prominent philtrum, anteverted nostrils, thin upper lip vermilion, and prefrontal edema, are reported. In two of the cases, the diagnosis was confirmed by clinical examination after birth. In the other, the diagnosis was made with noninvasive prenatal testing by detecting a novel mutation of the NIPBL gene in maternal blood. This report documents the importance of a focused examination of the fetal face in fetuses with abnormal upper limbs for the prenatal diagnosis of Brachmann-de Lange syndrome.