Circumscribed palmar and plantar hypokeratosis (CPPH) is a recently described skin condition with particular clinical and histopathological findings. The etiopathogeny of CPPH is still unclear, but an abnormal clone of keratinocytes has been postulated as the possible origin. CPPH has been considered an entity with no potential malignant transformation, but recently, it has been recognized in association with signs of actinic keratosis in the same biopsy. CPPH has never been recognized in association with porokeratosis. Although these entities seem to be different, a possible relationship between them might be suggested.