Focal acral hyperkeratosis (FAH) is a rare genodermatosis. FAH is usually observed in the second or third decades of life, and it is a type of palmoplantar keratoderma, a heterogeneous group of diseases characterized by abnormal incrassation of the palms and soles. Although it is an autosomal dominant inheritance, it could also be sporadic. It is also known as a rare variant of Costa's Acrokeratoelastoidosis (AKE). Its etiology is not known clearly and there is no effective treatment. In the present study, a 43-year-old recently diagnosed FAH case is presented due to its rarity and frequent confusion with AKE based on current literature