Cutis verticis gyrata (CVG) is a rare cause of acquired scalp thickening; in its primary form, the disease does not show any bone involvement. A 19-year old man had noticed a moderate thickening of the scalp at the age of 15. He progressively developed ridges and furrows in the occipital and parietal areas: after a four-year period, there were ten sagittal and irregularly parallel folds. There was neither pain nor pruritus, and the hair had a normal appearance. Complete neurological examination was unremarkable and the patient was of normal intelligence. All biological investigations, including STH and TSH levels, were normal. X-ray examination and tomodensitometry did not show any bone involvement. Our patient was treated by surgical reduction of the scalp areas involved under general anesthesia, with a very good result. This is a typical case of primary CVG. Differential diagnosis of primary cutis verticis gyrata comprises pachydermoperiostosis, cerebriform intradermal nevus and many secondary conditions associated with hypertrophy and folding of the scalp. A relatively simple surgical treatment results in a major reduction of the scalp thickening, and avoids maceration.