Darier's disease is a genetic disorder inherited as a autosomal dominant. The aetiology is unknown but electron microscopic studies suggest that the essential abnormality is a defect in the synthesis, organization, or maturation of the tonofilament-desmosome complex, which leads to abnormal keratinization. Reports of this condition in Africans is uncommon. We present here a case of Darier's Disease in a 70 year old Nigerian woman. As far as we know, it is the first reported case in a Nigerian. Clinical variants, complications, and differential diagnoses of Darier's disease are discussed.