Cerebral venous thrombosis (CVT) remains a diagnostic and therapeutic challenge for clinicians. Manifesting in a remarkably wide spectrum of symptoms and signs, CVT often presents in a misleading fashion—if unrecognized or misdiagnosed, it carries potentially fatal consequences. Visual loss is quite rare as the initial presentation of CVT and is typically a finding more frequent in chronic cases with associated papilledema on funduscopy Ferro, Lopes, Rosas and Fontes (Delay in Hospital Admission of Patients with Cerebral Vein and Dural Sinus Thrombosis. Cerebrovasc Dis 2005;19:152–6). We report a rare case of acute cortical blindness as the initial presentation of CVT in an 18-year-old female patient and review the current literature.