Introduction: Cerebral Venous Sinus Thrombosis (CVST) is a rare but potentially life-threatening cerebrovascular disorder in children. While inherited and acquired prothrombotic conditions are recognised risk factors, dyslipidaemia is rarely reported as a contributing factor in the paediatric population. Case presentation: A 12-year-old previously healthy boy , third born to non-consanguineous parents, presented with a 10day history of frontal headache worsening over three days, which was associated with 1-2 daily episodes of vomiting and posterior neck pain. He subsequently developed acute onset bilateral painless visual loss. On the second day of admission, he experienced right-sided focal seizures followed by deterioration in Glasgow Coma Scale (GCS). Paternal history of hyperlipidaemia with venous thrombosis was noted. Examination revealed an overweight child (BMI 26.3 kg/m2) with markedly reduced visual acuity . Other systemic and neurological examinations were unremarkable. Fundoscopy findings were suggestive of raised intracranial pressure. Lipid profile demonstrated elevated total cholesterol (284 mg/dL) and low density lipoprotein (LDL) (200mg/dL), with high density lipoprotein (HDL) 54 mg/dL and triglycerides 149 mg/dL. Other haematological and biochemical parameters were normal. CT cerebral venography revealed thrombosis in bilateral parasagittal frontal and parietal regions and left occipital region, with a small hemorrhagic focus in the left temporal region. Genetic analysis was deferred due to financial constraints. Therapeutic anticoagulation with enoxaparin,later transitioned to warfarin and statin therapy were initiated. Conclusion: This case highlights CVST as a rare presentation of familial hyperlipidaemia, emphasising early neuroimaging, prompt anticoagulation, cardiovascular risk assessment, and structured family screening to reduce morbidity and prevent future thrombotic events.