Background: Congenital melanocytic nevus (CMN) is melanocytic lesion present at birth and acquires a diameter equal to or bigger than 20 cm during adult life. Inspite of its rarity, this lesion is important because it is related to severe complications, like neurocutaneous melanosis, cutaneous or extra-cutaneous melanoma and its impact on the patient’s psychology, due to the aesthetic problems caused by it. Case Report: We hereby report a neonate with a giant congenital melanocytic nevus occupying an extensive area of the torso, accompanied by multiple satellite lesions in the limbs and the scalp. On ultrasound examination the brain proved to be normal, whereas MRI was not conducted, according to the parents’ wish. Conclusion: Occasioned by this incident, the importance of the proper evaluation and investigation is highlighted, aiming at the timely diagnosis for the detection of the early symptoms and/ or of melanoma.