Introduction Behcet’s Disease (BD) is manifested by a triad of relapsing hypopyon uveitis, aphthous stomatitis and genital ulcers.

We present the case of a school-aged boy with a Behçet-related acute cerebral venous thrombosis.

Material and method A 8 years old boy is admitted for acute and severe headaches with nausea.

Clinical examination notes a febrile child with marked aphtous mouth (with a history of genital ‘burns’).

ESR and CRP are mildly high, while cerebral angio-CT reveals a superior sagittal sinus occlusion.

Steroids and palliative management completely resolve these symptoms, while angio-MRI confirms the isolated thrombophlebitis.

Discussion The major manifestations of vascular Behcet Disease include venous occlusion, arterial occlusion and aneurysm formation.

Cerebral venous thrombosis (CVT) results in signs and symptoms of increased intracranial pressure, like for our child.

Steroids remain the mainstay initial treatment, with a particular attention to anticoagulation and adjunction/relay with colchicine.

Conclusion Cerebral venous thrombosis may reveal BD is associated with a good prognosis when treated promptly, specially in paediatric patients.