Background: Sturge Weber Syndrome is a sporadic neurocutaneous disorder of elusive etiology which is characterized by a vast continuum of manifestations ranging from neurological, cutaneous and ocular features. The main complications of the disorder include epileptic seizures, hemi paresis, and delayed neuropsychological development leading to poor quality of life. Case presentation: We present a 28 years old female of African descent who was seen at our neurology outpatient clinic in Dar es Salaam, Tanzania with a chief complaint of relapsing generalized motor seizures for the past 1 month. She had been on Anti-Epileptic Drugs since birth with poor control. Physical examination revealed an obese lady with Port Wine Stain appearance on the left half of the head, face, and neck. She had reduced visual acuity on the left eye, dysphasia with severe right-sided spastic hemi paresis. Her Computed Tomography films revealed extensive gyral and sub cortical calcifications seen on the left posterior cerebral parenchymal also involving the parietal-temporal lobes.