Background <p>Cerebral venous sinus thrombosis (CVST) is a rare type of stroke characterized by the formation of blood clots within the dural venous sinuses which has a high mortality rate. Despite its potential severity, cerebral venous sinus thrombosis secondary to nephrotic syndrome remains rarely reported and under-recognized in clinical practice.</p> Case presentation <p>We described a 20-year-old male who presented with a relapse of minimal change disease, complicated by extensive CVST. On the basis of anticoagulant therapy, his neurological condition deteriorated rapidly, manifesting as altered mental consciousness, progressive limb weakness, and new-onset seizures. Given the failure of conventional medical management, endovascular therapy of catheter-directed thrombolysis was performed. The patient’s neurological status improved dramatically within days.</p> Conclusions <p>This case demonstrates that cerebral venous sinus thrombosis should be considered in nephrotic syndrome patients presenting with even subtle or non-specific neurological symptoms. Patients with focal neurological deficits, altered mental status, new-onset seizures, or unusual headache should undergo prompt neurological evaluation and appropriate brain imaging, including computed tomography venography (CTV) or magnetic resonance venography (MRV) when CVST is suspected. In the specific setting of nephrotic syndrome, clinicians should maintain a heightened index of suspicion for CVST, even when initial neurological findings are subtle or non-specific. As illustrated by this case, early recognition and timely escalation to endovascular therapy can be life-saving when conventional anticoagulation fails to prevent neurological deterioration. Our patient achieved complete neurological recovery and sustained remission of nephrotic syndrome, underscoring the value of multidisciplinary collaboration and individualized treatment strategies.</p>