Background: Tuberous Sclerosis Complex (BourÂneville’s disease) is genetic disorder with autosomal pattern of heritence that affecting differentiation sellular in many organ such as brain, lungs, cardiac with varible severity. Birth incidence is estimated to be 1:6000. The purpose of this study was to reported a case of patients with Bourneville’s disease (tuberous sclerosis complex) in Moewardi Hospital. Also reported surveillance and complication in this patient.Case Presentation: A male, 18 yearsold, with seizures, onset from infancy, tonic secondary generalized seizure pattern simultaneously througÂhÂout the body. Neurologic status examiÂnation found right hemiparese and mental reÂtardaÂtion, Encountered in patients with skin disorders such as butterfly appearance with facial angiofibroma, forehead fibrous plaque dan sharÂgreen patch. Abnormalities also appeared in the oral mucosa as multiple papules. When psychÂological tests patient had difficulty following instructions. Psychiatric examination showed modeÂrate lower intelegence. EEG results slow waves at left parietal, there is severe electroÂfisioÂlogic abnormality considered structural lesiÂon in left parietal. CT showed multiple calciÂfied nodulÂes in subependymal right and left lateral ventriÂcle. MRI showed atrophy and left sclerotic hipÂpoÂcampus with glosis in sub cortex occipito left parietal. To overcome the seizure patients given Carbamazepine. There was inÂcreasÂing of transÂaminase enzim, liver suspect liver angioÂmyoÂlipoma.Conclusion: Based on clinical criteria for definite tuberous sclerosis complex patients met the criteria. The patients responded positively to carbamazepine that reduced seizure frequency. But somehow, patient's prognosis is worst. PreÂsence of intellectual impairment and neuroÂpsyÂchiatric problems is a potential poor epilepsy control, and liver disfunction so that the necessaÂry comprehensive management and surveillance involves other disciplines.Keywords:Tuberous sclerosis complex, conÂgenital disorder, epilepsy, mental retardationCorrespondence: Baarid Luqman Hamidi.Department of NeuroÂlogy, Dr. Moewardi Hospital/Faculty of MediÂcine, Universitas Sebelas Maret. Email: baÂarid@Âstaff.uns.ac.idIndonesian Journal of Medicine (2020), 05(01): 47-51https://doi.org/10.26911/theijmed.2020.05.01.07