Skew deviation is a vertical misalignment of the eyes caused by damage to prenuclear vestibular input to ocular motor nuclei. The resultant vertical ocular deviation is relatively comitant in nature, and is usually seen in the context of brainstem or cerebellar injury from stroke, multiple sclerosis, or trauma. Skew deviation is usually accompanied by binocular torsion, torticollis, and a tilt in the subjective visual vertical. This constellation of findings has been termed the ocular tilt reaction. In the past two decades, a clinical localizing value for skew deviation has been assigned, and a cogent vestibular mechanism for comitant and incomitant variants of skew deviation has been proposed. Our understanding of skew deviation as a manifestation of central otolithic dysfunction in different planes of three-dimensional space is evolving. The similar spectrum of vertical ocular deviations arising in patients with congenital strabismus may further expand the nosology of skew deviation to include vergence abnormalities caused by the effects of early binocular visual imbalance on the developing visual system.