Purpose: Superior ophthalmic vein thrombosis (SOVT) is an extremely rare condition. Few studies have been published about clinical aspects of this condition. In this study, we have studied the symptoms, underlying etiologies, treatment, pathogenesis and complication of the SOVT and we tried to classify it based on the etiology, treatment, and prognosis. Methods: We reviewed the patients’ data from a tertiary academic referral center. Each patient with SOVT was then reviewed for symptoms associated with SOVT, underlying etiology, treatment protocol, treatment response, complications, possible pathogens, and final outcome. Results: Twenty-four cases of SOVT were included in this study. Overall, 13 cases were diagnosed as right-sided SOVT, out of which, eight had simultaneous right-sided cavernous sinus thrombosis (CST). Eighteen cases were diagnosed to have left-sided SOVT, out of which, 11 had simultaneous left-sided CST. Conclusions: The SOVT can be secondary to different mechanisms. The SOVT secondary to trauma, recent surgery and coagulopathy are mostly non-aggressive, and can be managed by conservative therapy and anticoagulation. The SOVT in patients with orbital cellulitis, history of active sinusitis or paranasal sinus surgery are aggressive presenting with acute orbital swelling, abscess and visual loss. This type of SOVT can be complicated by extension to the cavernous sinus and intracranial structures. These patients require urgent antibiotics therapy and sinus surgery. The most severe type of SOVT is caused by mucormycosis which may also extend intracranially resulting in stroke and is often life-threatening.