Circumscribed acral hypokeratosis (CAH) is an idiopathic disorder characterized by a solitary, asymptomatic, well-defined, annular, erythematosus depressed lesion, with a slightly raised scaly border on the palm or sole. Histologically, there is sharp depression of cornified layer between the affected and non-affected areas, with the affected area having an abnormally thin stratum corneum and underlying epidermal changes. Here, we describe a case of CAH that was particularly unusual in a clinical feature. A 19-year-old woman presented with an asymptomatic erythematous patch on the left fifth finger which had waxed and waned for several years. Clinical examination revealed irregularly shaped linear scaly erythematous patch on the lateral to ventral area of the left 5th finger. A biopsy specimen showed abrupt decrease in the thickness of the orthokeratotic stratum corneum between the involved and uninvolved skin. About 8 months later, a similar lesion occurred on the left 4th finger. Skin biopsy resulted in the same finding as the previous one.