A rare harmful case of invasive melanocytic nevus in a child is described. In accordance with guidelines, independent method, histological and IHC analysis, MRI, consultations with neurologist and neurosurgeon are recommended. Giant congenital melanocytic nevi are rare. In some cases, such nevi may not just be an isolated lesion, but symptom of phacomatosis (neurocutaneous melanosis). Differential diagnosis is usually difficult due to gradual progression of disease. Primary diagnostic screening usually includes MRI of the central nervous system, histological examination of nevi and consultations with appropriate specialists.

Keywords. giant nevus, invasive nevus, melanocytic nevus, congenital nevus, giant nevus in children