A clinical case resembling cutis verticis gyrata due to a cerebriform intradermal nevus (CIN) is reported. Such a lesion is diagnosed on clinical and histopathological grounds. Some aspects are particularly discussed, such as complications of CIN, including the potential development of malignant melanoma, prognosis and different possibilities in the therapeutic approach. It is stressed that early diagnosis, broad surgical excision and plastic reconstruction are major issues. Different techniques of surgical extirpation and scalpflap reconstruction are presented.