Tuberous sclerosis is reported rarely and is associated with systemic lesions including central nervous system, skin, heart, eye and kidney. Approximately 5-15% of individuals with tuberous sclerosis will develope brain neoplasia, almost invariably subependymal giant-cell astrocytoma (SGCA). We experienced a case of SGCA with tuberous sclerosis operated by the transcallosal approach and report with literature review.