Circumscribed acral (or palmoplantar) hypokeratosis (CAH) was first described by Perez et al. in 2002. To date, several dozen of cases have been reported around the world. Despite its long course, typical clinical presentation and pathomorphological pattern, the diagnosis is challenging. Acral hypokeratosis is more common in women between the ages of 35 and 80. The etiology of the disease is poorly understood. Trigger factors for circumscribed palmoplantar hypokeratosis include repetitive trauma, burns, chronic mechanical effect, and disruption of cornification processes. Circumscribed palmoplantar hypokeratosis is also considered as a type of keratoderma associated with an aberrant mutant clone of epidermal cells or as a type of chronic non-inflammatory disorder of cornification processes in acral areas of the epidermis with involvement of the granular and horny layers. There’s some published evidence of HPV types 4 and 6 identified in foci of hypokeratosis. The clinical presentation includes a circumscribed area of the horny layer thinning. Lesions are commonly single, less commonly multiple, rounded or oval, pink, with a sharp outline, 1-4 cm in diameter. Peripherally, fragments of the intact horny layer are observed. A typical localization is the thenar and hypothenar eminence more often in the right hand, but localization of lesions on the phalanges of the fingers has also been reported. The disease has a chronic course lasting from 2 to 30 years. The differential diagnosis includes Bowen’s disease, porokeratosis, palmoplantar psoriasis, pitted keratolysis, glabrous skin mycosis, and secondary syphilis. The pathomorphological study shows a sharply delineated zone of stratum corneum thinning with hypogranulosis in the central zone and perivascular lymphocytic infiltration of the superficial regions of the dermis. The disease is asymptomatic and difficult to manage and treat. The most effective treatment is surgical excision, which can only be applied to small lesions.