Neurocutaneous melanosis (NCM) is a rare phakomatosis characterized by proliferation of melanin-producing cells in both the skin and the brain. In this study, we describe the clinical and pathologic features of NCM in a 4.5-year-old female cynomolgus macaque. Histopathologically, skin lesions showed foci of nests and cords of pigmented cells in the dermis similar to blue nevi in humans. In the brain, focal pigmented cell infiltration was observed in the connective tissue under the leptomeninges and in the brain parenchyma. The pigmented cell was moderately reactive with a pan-melanoma antibody (melanoma(pan)) in the skin. In the brain, the pigmented cell was moderately to strongly positive for melanoma(pan) in subleptomeningeal areas and in the cerebral cortex. Melanosomes were observed in pigmented cells in the brain by electron microscopic examination. Based on the histologic, immunohistochemical, and electron microscopic results, the diagnosis of NCM was made. This case is possibly the first report of the condition in animals.