Torticollis is an abnormal head posture caused by ocular or non-ocular factors. Ocular torticollis (OT) is a compensatory head posture adopted to avoid double vision or dampen nystagmus. In this review, we argue, that causes for OT comprise a wide range of congenital or acquired ocular conditions. These patients are at risk of being mis-cat-egorised as having non-ocular torticollis, which may delay treatment and cause amblyopia in children. OT can be symptomatically relieved by prismatic correction incorporated in the patient's glasses or treated by strabismus surgery, which also improve ocular motility.