Cutis verticis gyrata (CVG) is an abnormality of the scalp characterized by
the formation of furrows and folds which cannot be flattened by traction o
r pressure. Primary and secondary forms of CVG have been described. We repo
rt on a patient affected by cutis verticis gyrata, mental regression and Le
nnox-Gastaut syndrome (LGS). Serum hormonal levels, karyotype and X fragile
studies were normal. Magnetic resonance imaging of the brain showed only a
trophic changes. The etiology of primary CVG remains unknown as does its re
lation with LGS.