Hemifacial spasm (HFS) is characterized by involuntary, irregular contraction of the muscles innervated by one facial nerve. Usually, it is caused by facial nerve injury either due to microvascular compression or a posterior fossa tumor, but it also occurs without apparent cause. It is rare in children; no congenital cases have yet been reported. We report the first case of congenital HFS in a term newborn delivered by forceps after a normal labor. Multimodal evoked potentials, electroencephalogram, computed tomography of the petrous bone, as well as brain magnetic resonance imaging and angiography disclosed no abnormalities. Serial neurodevelopmental examinations and video recordings performed until 8 months of age documented a normal neurodevelopmental status and a tendency for spontaneous diminution of the HFS. An intrauterine facial nerve injury as the causative factor of HFS, being responsible for its benign course, is proposed.