This review summarizes the current knowledge regarding the functions of PGC-1α in the brain and its role in neurological diseases, such as intracerebral hemorrhage (ICH), ischemic stroke, Alzheimer’s disease (AD), Parkinson’s disease (PD), Amyotrophic lateral sclerosis (ALS), and Polyglutamine (PolyQ) diseases. The gene discussed is PPARGC1A; the disease is ischemic stroke.