Objective: Thrombosis is a common complication of nephrotic syndrome (NS). However, cerebral venous sinus thrombosis (CVST) secondary to NS is rarely reported. Here we report a case series study of 5 cases of CVST with NS, so as to make a better understanding and management of this disorder. Methods: A retrospective study was performed in 5 consecutive patients with CVST in combination with NS between 2009 and 2015. The clinical manifestations, laboratory and radiological findings, treatment, and clinical outcomes were analyzed. Results: This cohort of case series consists of 1 woman and 4 men, aged 16-49 years. All patients complained initially of an acute or subacute headache. CVST attacked during NS occurrence in 3 patients, and during NS recurrence in 2 patients. The median duration of signs and symptoms prior to clinical diagnosis and treatment was 12.80 +/- 7.53 days. In all patients, it was magnetic resonance venography that detected the thrombosis in the cerebral venous sinus, with the most common site of CVST to be the superior sagittal sinus (5 of 5 patients). Two or more segments of sinus were involved simultaneously in 4 patients. The treatment of CVST in NS involved therapy of CVST in the general population. All the 5 patients had full recovery, and no one relapsed with a follow-up of 26.60 +/- 29.75 months. Conclusions: NS may be one of the important etiologies of CVST. When patients with NS had progressing headache, seizure, or other unexplained neurological symptoms, CVST should be considered.