A case of cerebriform melanocytic intradermal nevus is presented, beca
use of its extreme rarity, familial characteristics, and the good resu
lt obtained with total excision and skin grafting. The treatment is of
interest because these lesions may become malignant or infected, and
there can be aesthetic problems which could result in behavioral diffi
culties and the patient feeling socially rejected. We insist on the im
portance of following a systematic study of these patients, and possib
le extracutaneous lesions must be ruled out. The cerebriform intraderm
al nevus is a benign tumor, which may appear at birth or at an early a
ge. It is located on the scalp, and its external appearance is similar
to that of the brain.