Focal acral hyperkeratosis is clinically identical to acrokeratoelastoidosis but histologically lacks the elastorrhexis which is a feature of acrokeratoelastoidosis. We present a case of focal acral hyperkeratosis in a 50 year old woman. About 20 years ago, a few pinhead sized papules developed on radial side of index fingers. Thereafter size and number of papules have been gradually increased to cover entire hands sparing palms and dorsa of feet. The histopathologic study showed characteristic findings of focal acral hyperkeratosis. After treatment with etretinate, the size and thickness of lesions were reduced.