Purpose: To provide a comprehensive review on different characteristics of abnormal head postures (AHPs) due to different ocular causes, its measurement, and its effect on facial appearance. Methods: In this review article, PubMed, Scopus, and Google Scholar search engines were searched for the scientific articles and books published between 1975 and September 2020 based on the keywords of this article. The selected articles were collected, summarized, classified, evaluated, and finally concluded. Results: AHP can be caused by various ocular or nonocular diseases. The prevalence of ocular causes of AHP was reported to be 18%–25%. 1.1% of patients presenting to ophthalmology clinics has AHP. The first step in evaluating a patient with AHP is a correct differential diagnosis between nonocular and ocular sources by performing comprehensive eye examinations and ruling out other causes of orthopedic and neurological AHP. Ocular AHP occurs for a variety of reasons, the most important of which include nystagmus, superior oblique palsy, and Duane's retraction syndrome. AHP may be an essential clinical sign for an underlying disease, which can only be appropriately treated by the accurate determination of the cause. Long-standing AHP may lead to facial asymmetry and secondary muscular and skeletal changes. Conclusion: In conclusion, a proper differential diagnosis between nonocular and ocular causes, knowledge of the different forms of AHP and their measurement methods, accurate diagnosis of the cause, and proper and timely treatment of ocular AHP can prevent facial asymmetry and secondary muscular and skeletal changes in the patients.