The authors describe two cases of cerebral venous thrombosis (CVT) in patients with nephrotic syndrome. The main clinical features of CVT were persistent headache, hemiparesis, and seizure, and the diagnosis was based on magnetic resonance imaging and magnetic resonance angiography. Both showed acquired deficiency of free protein S. The neurologic symptoms remained stationary in the first patient, who received no anticoagulation therapy, but resolved rapidly in the second, treated with intravenous heparin and supplemented with fresh frozen plasma. CVT should be suspected in patients with nephrotic syndrome who present with symptoms of intracranial hypertension or any focal neurologic deficit.