Congenital primary cutis verticis gyrata

Congenital cutis verticis gyrata (CCVG) affects 1 in 100？000 males and 0.026 in 100？000 females. Characterized by thick skin folds and grooves on the scalp resembling the outer surface of the brain (1), CCVG can present in primary and secondary forms (2). The inheritance of this condition remains controversial (3). While primary CCVG has been associated with chromosomal abnormalities as Noonan and Turner syndromes, there are no many reports of CCVG in a patient with Trisomy 21 (4). Timely recognition of CCVG is important as this condition is linked to serious neurological and ophthalmological anomalies