The authors present 2 months old child with giant congenital melanocytic naevus of thoraco-lumbosacralis region and numerous congenital pigmented nevi disseminated on the skin of the whole body, slight right hemiparesis and epileptic seizures. Magnetic resonance imaging (MRI) of the head performed at the age of 2 months showed melanin deposits in the temporal lobes. The diagnosis of neurocutaneous melanosis was made on the basis of clinical picture.