We used proton magnetic resonance spectroscopy to examine resonances representing metabolites containing N-acetyl (NA) groups (predominantly N-acetyl aspartate), choline, and creatine within a large left-hemispheric gray matter heterotopia (GMH) in a 35-year-old man with corpus callosum agenesis. In contrast to normal brain tissue, including gray matter regions, heterotopic gray matter was characterized by relatively increased choline and creatine resonances and a normal NA signal. These data suggest increased cellular activity or persistent immature neuronal tissue in GMH relative to unaffected tissue.