Circumscribed palmoplantar hypokeratosis (CPH)- characterized by an abrupt thinning of the corneal layer- was first reported in 2002. With regard to clinical appearance, it is often described as a well-demarcated, round to oval-shaped depression with an erythematous center and a slightly scaly border. CPH develops in naturally hyperkeratotic areas such as the palms and soles. Some studies suggest that the incidence of CPH may be underestimated because it is often asymptomatic and can be misdiagnosed as another disease. Porokeratosis and Bowen’s disease are also included in the differential diagnosis of CPH. To identify CPH, skin biopsy of the margin is recommended, which shows a sharp step-off of the stratum corneum. A 62-year-old woman presented with a 6-mm, solitary, well-demarcated, slightly depressed, erythematous plaque with a scaly border on the radial aspect of the second metacarpal area of the left hand. The lesion was initially detected 3 years previously, but was asymptomatic; hence, she did not visit a clinic. Moreover, she did not have any history of trauma on the area and reported no change in size. To definitively diagnose and treat the lesion simultaneously, excisional biopsy was performed. Herein, we report a case of CPH for an educational purpose.