Pityriasis rubra pilaris (PRP) is a chronic, inflammatory, papulosquamous disorder of keratinization of unknown etiology. It appears both in hereditary and in acquired form. According to the classification by Griffiths, there are six types of PRP: classical adult (type I), atypical adult (type II), classical juvenile (type III), circumscribed juvenile (type IV), and atypical juvenile (type V). Also, HIV-associated type VI has been described. Typical lesion is follicular hyperkeratotic papule. Spearing “islands” of not affected skin are a pathognomonic sign. Palms and soles are almost always involved, often showing an orange color, with palmoplantar waxy keratoderma. Histology is not confirmatory. PRP should be distinguished from psoriasis and other papulosquamous skin disorders. Treatment is topical or systemic. Retinoids are first-line treatment. Biologics are a new class of available medications for the off-label treatment of PRP, basically used with success in psoriasis.