The physiologic ocular tilt reaction (OTR) consists of head tilt, biocular conjugate torsion, and skew deviation. The utricle otolith, external ocular skew muscles, brainstem, and cerebellum collectively mediate it. Peripheral and central lesions lead to pathological OTR. The aim of the paper is to inform clinicians of the importance of OTR, which should not be misdiagnosed as ocular muscle palsy, semicircular canal palsy, or torticollis when peripheral or central lesions are considered. Ophthalmologic strabismus exams, otoneurology, and imaging studies are helpful in finding these lesions. Finally, a flow sheet is included.