Background/Aims: The aim of this study is to highlight the variable clincoradiological spectrum of isolated cortical vein thrombosis (ICoVT), which seems to remain a challenge to clinicians. Cases Reports: We reported 3 patients with this diagnosis. One presented with only an epileptic seizure, one with worsening headache, seizures, mental disorder, speech disturbance and right-sided weakness, and the other with seizures and fluctuating paralysis in her left-sided limbs. Brain images were manifested with a strand-like abnormal signal, a large hemorrhagic infarction and a continuously enlarged space-occupying massive edema, respectively. Conclusions: Neurologic features and brain imaging of ICoVT are highly variable, which might be partly responsible for the underestimation of ICoVT. Clinical diagnosis should probably be evoked more often. Copyright (C) 2013 S. Karger AG, Basel