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Abstract:  The potential for malignant transformation of a giant congenital melanocytic nevus (GCMN), although rare, should be considered, especially in proliferative lesions that have nodules on their surface, as they can clinically and histologically mimic melanoma and generally have a poor prognosis. We present the case of a newborn boy with a giant nevus of uneven blackish color on the back with variable degrees of intensity of pigmentation, almost entirely covered by nodules of large and variable sizes, soft texture, and a tendency to ulcerate and bleed. The unusual clinical and histologic appearance made the case particularly interesting and featured a not well-defined biologic behavior, thus deserving a radical surgical approach to fulfill diagnostic and therapeutic aims.