Subependymal giant cell astrocytoma (SEGA) is a unique brain tumor that constitutes one of the major diagnostic criteria of tuberous sclerosis complex (TSC). It rarely occurs de novo after adolescence. SEGA tends to originate from the ventricular walls, provoking obstructive hydrocephalus, raised intracranial pressure, and papilledema with plausible visual problems. We present a case of large SEGA in a 33-year-old-TSC patient with a higher-than-expected age for the first presentation. His chief complaint was visual acuity deterioration combined with headaches. Microsurgical removal of the tumor was carried out. Obstructive hydrocephalus and papilledema resolved after treatment. Although SEGA-TSC is considered a tumor of children and adolescents, it can present for the first time in adulthood. In TSC patients, periodic imaging follow-up is recommended and any visual symptoms should prompt exclusion of intracranial tumors.