Association of leukoencephalopathy, cerebral calcifications, and cysts (LCC) is a rare disorder that was recently described. To our knowledge, only 2 reports, including 3 patients in each, have been published in the literature to date. Herein, we report a 19-year-old man with LCC who had neurological symptoms beginning in late adolescence. Clinically, he had rare convulsive seizures, slowly progressive pyramidal symptoms, and normal intelligence. On radiological examination, there were progressive calcifications in the basal nuclei and the cerebral white matter, as well as parenchymal cysts and diffuse abnormal signals of the white matter on T2-weighted sequences on MR imaging. On histopathological examination, angiomatous changes and secondary gliosis were demonstrated.