Cerebral venous thrombosis (CVT) is a rare cause of stroke that can lead to cerebral edema, intracranial hemorrhage, and severe neurological complications. Autonomic nervous system dysfunction following acute brain injury may trigger life-threatening cardiac arrhythmias.This case report is based on retrospective analysis of clinical data, imaging studies, laboratory findings, electrocardiographic monitoring, and neurological investigations collected during hospitalization.This case highlights the association between CVT, refractory status epilepticus, and malignant cardiac arrhythmias in the absence of primary cardiac pathology.Severe neurological conditions may disrupt cardiac rhythm regulation. Early recognition of atypical neurological presentations and close cardiac monitoring are essential, including in patients without known heart disease.A 54-year-old man was admitted after a fall following a generalized tonic–clonic seizure. He rapidly developed refractory status epilepticus requiring mechanical ventilation. During hospitalization, he experienced ventricular fibrillation successfully treated with defibrillation. Etiological investigations were initially inconclusive. Cerebral CT angiography later revealed thrombosis of the left sigmoid sinus extending to the ipsilateral internal jugular vein.This case illustrates the diagnostic and prognostic challenges of cerebral venous thrombosis presenting with refractory status epilepticus. CVT may initially mimic other neurological emergencies, leading to delayed diagnosis when early neuroimaging is inconclusive. The occurrence of malignant ventricular arrhythmia in this patient highlights the impact of severe neurological injury on autonomic nervous system regulation. Intense sympathetic activation during refractory seizures and cerebral edema may precipitate life-threatening cardiac rhythm disturbances, even in the absence of structural heart disease. This neurocardiac interaction remains under-recognized and may contribute to unexpected mortality.CVT may present with refractory seizures and severe autonomic dysfunction leading to malignant arrhythmia. Continuous cardiac monitoring should be systematically considered in patients with severe neurological involvement.