Facial asymmetry present only on crying has been described as a separate entity and termed asymmetric crying facies. The cause of the facial asymmetry in this disorder is congenital absence or hypoplasia of the depressor anguli oris muscle at the corner of the mouth. This defect is associated at times with major congenital anomalies, most commonly in the cardiovascular system. Chromosome 22q11 microdeletions in cases with ACF have been reported. We report a newborn infant who had ACF associated with a couple of primary mandibular central incisor teeth and chromosome 22q11 microdeletion. This clinical sign in association with ACF has not been previously described.