Neurocutaneous melanosis is often associated with melanoma of the central nervous system. Due to the inconspicuously looking cutaneous melanosis additional examinations are often not performed. We describe a patient with cutaneous melanosis who presented with neurological symptoms due to a large primary meningeal melanoma. The diagnosis of neurocutaneous melanosis was made. This case is an illustration of melanoma development in the central nervous system in a patient with cutaneous melanosis. This phenomenon should be kept in mind when observing patients with such skin lesions, even if they are of minor extent.