Cutis verticis gyrata (CVG) is a descriptive term for a scalp condition that is convoluted folds and deep furrows that resemble the surface of the cerebral cortex. It is categorized by the underlying etiology, as primary essential, primary non-essential and secondary. Alopecia areata (AA) is a common, organ specific autoimmune disease, and most AA cases are sporadic. There is clearly a strong genetic component. There is no established relationship between CVG and AA. We report one case which was affected with essential primary CVG and alopecia areata, and suggest a possibility of genetic association between CVG and AA, possibly both being related to mutations in the fibroblast growth factor receptor 2 (FGFR2).