Acrokeratoelastoidosis is a genodermatosis characterized by firm papules or plaques on the sides of the hands and feet. Although poorly understood, the lesions may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. In this report, we will present a patient with this rare condition and review the clinical and histopathologic features, cause, and differential diagnosis. (J Am Acad Dermatol 2002;47:448-51.)