Abstract: Congenital melanocytic nevi (CMN) are benign skin lesions present in some newborns. We describe a newborn boy with a giant CMN on the scalp. It appeared as an atrophic patch with a central nodule that was suggestive of a rapidly involuting congenital hemangioma or some other vascular tumor. Histopathology confirmed the diagnosis of CMN. Magnetic resonance imaging was normal, excluding leptomeningeal melanocytosis. We did not find any other cases of CMN with this presentation in the literature.