A 7-year-old Chinese boy with steroid-resistant nephrotic syndrome developed thalamic stroke secondary to straight sinus thrombosis. He was hospitalized due to status epilepticus and coma. The child recovered after treatment by low-molecular-weight heparin (LMWH) and warfarin. This case highlights the importance of magnetic resonance imaging with venography in the early diagnosis of cerebral sinus thrombosis (CST) in nephrotic children and the effectiveness of anticoagulation therapy in improving the neurological outcome.