An abnormal head position (AHP) refers to a condition where the head is deviated from the usual, upright posture considered normal. AHPs can manifest as the chin being raised or lowered, the head tilted to the right or left, the face turned to either side, or a combination of these movements. Patients with AHP may present to ophthalmologic clinics; however, there are several etiologies for AHP that may not be commonly recognized by ophthalmologists. Key words from this article were searched in PubMed, Scopus, and Google Scholar search engines from 1975 to December 2023. Various etiologies were identified, evaluated, summarized, and then categorized. The maintenance of a normal head posture during our daily activities relies on the complex interaction of different parts of the brain, with the encoding of related sensory inputs occurring in the vestibular nuclei. Abnormal head posture can stem from a variety of etiologies, including ocular, neurological, orthopedic, otolaryngological, gastroenterological, and other factors. This review provides a comprehensive overview of the different characteristics of AHP based on its etiology. Lack of awareness regarding the wide spectrum of causes may lead to patients undergoing unnecessary extensive workups. Conversely, failure to recognize potentially life-threatening causes may result in adverse outcomes for the patient.