Lentigines are usually flat, pigmented macules on the skin and mucosa. These lesions are characterized by mostly smaller than 0.5 cm, irregular borders and different shades of brown and black. They can be in the form of circumscribed or widespread lesions, and occur either as isolated lesion or as a part of some congenital syndromes. The speckled lentiginous nevus is also named as Nevus Spilus which is a peculiar acquired lentiginous nevus, which can be divided into two distinct clinicopathological variants as macular and papular. On the other hand, centrofacial lentiginosus is a special form of congenital lentiginosus which is inherited as autosomal dominant, and is usually associated with some neurological and psychiatric findings. We report a 43-year old woman with a giant maculo-papular speckled lentiginous nevus associated with a different and non-syndromal isolated centrofacial lentiginosus. To the best of our knowledge this association has not been reported previously.