ABSTRACT
Sturge Weber syndrome is the third most common neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis. Three distinct types were identified. Type 3 with leptomeningeal involvement alone is the rarest among other types. The reported case is a 21-years-old female patient without any known chronic disease. She admitted to the emergency department after visual symptoms and headache, followed by generalized tonic clonic seizure. EEG of the patient showed left occipital seizure activity. The contrast enhanced magnetic resonance imaging (MRI) showed left occipital leptomeningeal angioma. Digital substraction angiography (DSA) revealed minimal blushed contrast enhancement on late venous phase and lack of superficial cortical veins. Her focal seizures were under control with levatiracetam and lacosamide treatment. The reported case is unique because of the late onset presentation with focal seizure without mental retardation.