Background: Sturge Weber syndrome (SWS) is rare congenital developmental disorder associated with   a facial port-wine birthmark, a vascular malformation of the brain and glaucoma. SWS is a multigenetic disorder caused by mutation in the GNAQ gene. This gene provides instructions for making a protein called guanine nucleotide-binding protein G(q) subunit alpha (G?q). 
Objective: To report a case of Sturge Weber syndrome (SWS) in an eight-month old girl.
Case: an eight-month old girl was admitted with history of seizure, weakness of right sides of the body, and reddish rash on the left face since birth. Physical examination showed compos mentis, port wine stain, increased intraocular pressure in left sides, right hemiparesis extremities. There was spastic and increase tone in right side, and delayed fine and gross motor neuron. Hb 9.3 g/dl WBC 10.200/ul, MCV 82 MCH 27pg, PLT 631.000/ul. Head CT scan showed left frontoparietal lobe angiomatosis calcified lesions accompanied by suspected AVM and cortical atrophy Sturge weber syndrome. EEG from interictal epileptiform discharge (IED): Sharp wave continue left frontal + temporoparietal bilateral. Diagnosis based on 3 major clinical criteria, port wine stain, leptoangiomatosis, and glaucoma. After treatment with anticonvulsan, timolol maleat 0.25%, propanolol, iron supplement and medical rehabilitation, the patient's condition improved. 
Conclusion :  A case of SWS has been reported in an eight-month old girl. Diagnosis was based on clinical symptoms, physical examination, radiology, EEG and laboratory finding. The prognosis was dubia