Superior ophthalmic vein (SOV) thrombosis is an uncommon orbital pathology that can present with sudden onset proptosis, conjunctival injection, and visual disturbance. SOV thrombosis is frequently secondary to a cavernous sinus pathology. A 32-year-old female with a known history of autoimmune hemolytic anemia presented with sudden painful proptosis left eye, and on imaging, she was found to have SOV thrombosis without cavernous sinus involvement. She was diagnosed with unilateral isolated SOV thrombosis and was managed conservatively. A careful history and clinical evaluation can help diagnose such rare disorders and initiate appropriate therapy.