Mitochondria are the center of intracellular energy metabolism. Mitochondrial dynamics refers to the dynamic process of mitochondrial fusion and fission, which plays an important role in maintaining mitochondrial homeostasis and central nervous system function. Optic atrophy 1 (OPA1) is a key factor involved in mitochondrial dynamics. OPA1 acts by regulating mitochondrial fusion and fission, reducing oxidative stress, inhibiting apoptosis, and promoting mitochondrial autophagy, to maintain the dynamic changes in mitochondrial quantity, structure, and biological function. Numerous studies have shown that OPA1-mediated mitochondrial dynamics plays an important role in ischemic stroke, Alzheimer’s disease, Parkinson’s disease, spinal cord injury, multiple sclerosis, and other central nervous system diseases. Here we review the regulatory mechanism of OPA1 in terms of mitochondrial dynamics and the important role of mitochondrial function mediated by OPA1 in central nervous system diseases, to provide new ideas for clinical treatment.