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Cerebriform giant melanocytic nevus of the scalp is an extremely rare malformation. Clinical appearance with maceration and fetor within the crypts and the risk of malignant transformation may require surgical therapy. We report two cases with different methods of surgical management. A 27-year-old woman noticed a swelling of the parietal scalp that developed over a period of 4 years into a gyrus-like tumor measuring 12 cm × 18 cm. The crypts between the gyri could not be inspected. Serial excisions under subcutaneous infusion anesthesia were performed to reduce the size of the nevus and to flatten the surface of the scalp. The second patient, a 26-year-old man, demonstrated a giant 10 cm × 10 cm cerebriform nevus on the occiput. The nevus also contained areas of fetid maceration. After implanting a tissue expander under general anesthesia the nevus was excised. The defect was closed using a rotation flap.