Recently, the occurrence of pityriasis rubra pilaris (PRP) has been reported in patients with HIV infection. It presents different clinical features, and has a poorer prognosis, than the classical adult type of PRP. We report the occurrence of severe PRP in an HIV-infected patient, and review the previously reported cases of this association. We propose the designation of a new category of PRP (type 6), characterized by the presence of HIV infection, usually without immunosuppression, a poor prognosis and response to treatment, and the development of nodulocystic and lichen spinulosus lesions.