Chorea-acanthocytosis is 1 type of neuroacanthocytosis that is a group of rare, hereditary neurodegenerative disorders. We presented a brain FDG PET finding of a 31-year-old woman with chorea-acanthocytosis. The images demonstrated significant hypometabolism in bilateral caudate nuclei and putamina. The finding of FDG PET is more prominent than that of MRI. Another interesting observation is the mildly increased FDG uptake in pituitary gland, although its relationship with the disease is unclear.