Cutis verticis gyrata in a neonate

The primary form of cutis verticis gyrata, a rare skin lesion, is described for the first time in a neonate in whom it was associated with other congenital anomalies. The combination of a histologically normal skin biopsy from the lesion and neurologic deficit supported the diagnosis; follow-up at 7 months of age showed persistent hypotonia and significant developmental delay. Differentiation of primary and secondary forms of cutis verticis gyrata and currently recognized etiologies are also briefly reviewed.