We report a rare case of subcutaneous neurofibromas developing within a giant congenital melanocytic nevus in a four-year-old patient with Neurofibromatosis 1. Interpreting histopathological findings within a clinical context is critical to finalize a diagnosis when the differential in such patients include lipoma, neurotized melanocytic nevi, neurofibromas, or proliferative melanocytic nodules in a congenital melanocytic nevus. Although uncharacteristic in early childhood, neurofibromas can appear earlier in life. In our case, histopathological evaluation of the skin biopsy specimens revealed neurofibromas, and no evidence of malignancy.