Circumscribed palmar hypokeratosis is a distinctive disorder characterized by circular area of erythematous depressed skin involving the thenar and hypothenar regions of the palm. We present the case of 66-year-old man with circumscribed palmar hypokeratotic lesion. He developed an acquired, solitary, circumscribed, erythematous, depressed skin on the right palm. Histopathologic study demonstrated a depression of the epidermis, with a sharp stair between normal and involved skin. The epidermis covering the depression showed markedly thinner homy layer and slightly diminished granular cell layer when compared with adjacent noninvolved skin. Serial section failed to demonstrate cornoid lamellae. To our knowledge, this distinctive entity has been previously reported in Korea.