Cutis verticis gyrata, underrecognized neurocutaneous syndrome

I report two patients with cutis verticis gyrata. Characteristic of this neurocutaneous syndrome is the scalp tissue folds that generally run in an anterior-posterior direction. Although the etiology of the scalp abnormality is unclear, patients may have associated static encephalopathy, seizure disorder, or minor ocular abnormalities. The prognosis is benign. Other organ systems are spared and there is no malignant transformation of scalp tissue or brain parenchyma.