Torticollis can arise from nonocular (usually musculoskeletal) and ocular conditions. Abnormal head position for ocular reasons is usually assumed in order to maintain binocularity and/or to optimize visual acuity. A variety of conditions may be responsible. The sensory organ of position sense, the labyrinth, lies within the inner ear and it relates to eye movement in both a dynamic and static fashion. The Bielschowsky head tilt test is based on the functioning of the otolithic apparatus and is the primary test in evaluating abnormal head position. Treatment is usually surgical, with extent and location dependent upon on the underlying cause. While corrective surgery has usually been done on an elective basis for cosmetic purposes, there is evidence that uncorrected torticollis can cause musculoskeletal problems. This review describes the physiological bases for ocular torticollis and the diagnosis and treatment of its various underlying causes.