Isolated superior ophthalmic vein (SOV) thrombosis is a rare condition usually related to inflammation of the orbit or paranasal sinuses. Patients present with acute orbital signs, including proptosis, ophthalmoplegia, globe dystopia, and periorbital edema, and may have diminished vision secondary to optic neuropathy. SOV thrombosis is typically seen in the setting of septic cavernous sinus thrombosis, and antimicrobial therapy is the treatment of choice. We herein report what may be the first case of isolated SOV thrombosis related to hypercoagulability in a patient with cancer who was receiving antifibrinolytic and thrombopoietin receptor agonist medications.