Porokeratosis plantaris palmaris et disseminata is a rare variant of porokeratosis. A 37-year-old patient has been affected for about 2 years by mildly discornforting numerous, 1-3mm sized, keratotic lesions of the trunk and limbs, especially on the palms and soles. Histopathologically the diagnosis of porokeratosis was established by demonstration of a cornoid larnella. The family tree of the patient is compatible with autosomal dominant inheritance. The patient has been treated with topical 5-fluorouracil with little auccess.