Isolated cortical vein thrombosis (ICVT) is extremely rare. Only single case or small series of ICVT have been reported; clinical details are still uncertain. We report a case of isolated superficial sylvian vein thrombosis with exceedingly long cord sign. A 14-year-old female with severe sudden onset headache visited our hospital. Fluid attenuated inversion recovery and echo-planar T2(*) susceptibility-weighted imaging (T2(*)SW) showed a long cord sign on the surface of the sylvian fissure. The patency of dural sinuses and deep cerebral veins were confirmed by magnetic resonance venography (MRV), and diagnosis of ICVT was made. She recovered completely without anticoagulant agents. To clarify the clinical characteristics of ICVT, we reviewed 51 ICVT cases in the literature. In many cases, T2(*)SW was the most useful examination to diagnose ICVT. In contrast with general cerebral venous thrombosis, MRV and conventional angiography were either supporting or useless. Anastomotic cortical veins were involved frequently; symptoms of gyri around the veins were common. It also suggested that ICVTs of the silent area might have been overlooked because of nonspecific symptoms, and more patients with ICVT may exist. In cases involving patients with nonspecific symptoms, the possibility of ICVT should be considered.