The current data on various aspects of Brown syndrome are limited and sporadic. This review provides a coherent and comprehensive review of basic features, etiology, classification, differential diagnosis, and different management strategies of patients with Brown syndrome. In this topical review, PubMed, Scopus, and Google Scholar search engines were searched for papers, published between 1950 and January 2023 based on the keywords of this article. The related articles were collected, summarized, categorized, assessed, concluded, and presented. Brown syndrome is identified by restricted passive and active elevation of the eye in adduction. The condition is divided into congenital and acquired causes. The clinical features result from a restricted motion of the superior oblique tendon sheath through the trochlea while trying to look up in adduction. The newest explanation of the underlying pathophysiology has been explained as the presence of a fibrotic strand in the superior oblique muscle tendon with variable insertion sites which creates various elevation deficits seen in Brown syndrome. The most common clinical features include the presence of an abnormal head posture, V-pattern strabismus, and hypotropia in the primary position. Management of Brown syndrome includes watchful observation, surgical, and non-surgical procedures. Some cases might resolve spontaneously without any intervention; however, some acquired cases might require systemic and/or intra-trochlear steroid administration to treat the underlying causes. Surgical procedures such as superior oblique tenectomy and using a silicon tendon expander are indicated in the presence of hypotropia and significant abnormal head posture in the primary position.