Limited scleroderma, also termed CREST syndrome (calcinosis cutis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), is a form of scleroderma which is an autoimmune connective tissue disease. Neurological involvement is less common than other system involvements in scleroderma. Epileptic seizure is a rare neurological complication. Gyral enhancement is not an expected finding during disease. Gyral enhancement is contrast enhancement of superficial brain parenchyma and it usually occurs as a result of vascular, inflammatory, infectious and neoplastic processes. A-sixty-seven-year old female patient, who was diagnosed as CREST syndrome for three years was admitted to our hospital. She had an epileptic seizure three days ago and behavioral changes after the seizure. There was not epilepsy diagnosis in her medical history. In neurological examination; she was apathic and deep tendon reflexes were hyperactive, Hoffman’s and Babinski reflexes were positive on the right side of her body. In laboratory studies; anti-nuclear antibody was positive at 1/1000 and anti-centromere antibody was positive. On contrast-enhanced cranial magnetic resonance imaging (MRI); gyral enhancement was observed in the left parieto-occipital region. Cerebrospinal fluid tests were within normal limits. Electroencephalography demonstrated diffuse cerebral dysfunction. We administrated antiepileptic drug to the patient during hospitalization. On the follow up, apathy and restriction of the cooperation were improved. Epileptic seizure did not recur. After ten days, in the control cranial MRI, gyral enhancement was reduced significantly. Neurological involvement in CREST syndrome is rare and epileptic seizures have been appeared in a limited number in the literature. Inflammatory and vascular processes can cause gyral enhancement, but also it must be kept in mind that gyral enhancement can be observed transiently after epileptic seizure