A 24-year-old woman presented with asymptomatic hyperpigmented bilateral patches on her temples, eyelids and forehead since birth. Furthermore, the patient had a congenital grey patch, compatible with Mongolian spot, on her buttock. She had no vascular or other cutaneous lesion. Histopathologic examination revealed bipolar dendritic melanocytes dispersed in a ribbon-like pattern between the collagen fibers and around the neurovascular bundles of the dermis. As far as we know, this is the first case of bilateral “congenital” nevus of Ota in association with a Mongolian spot reported in a patient.