Many cutaneous diseases have been reported in patients with human immunodeficiency virus infection. We report two patients with pityriasis rubra pilaris and human immunodeficiency virus infection. In one patient, the onset of pityriasis rubra pilaris preceded the discovery of human immunodeficiency virus infection. In the second patient, the onset of pityriasis rubra pilaris occurred shortly after the patient tested positive for human immunodeficiency virus infection. Both patients had a severe form of pityriasis rubra pilaris, and both had a minimal therapeutic response to etretinate.