The development of pityriasis rubra pilaris (PRP) in three patients with human immunodeficiency virus (HIV) infection is described. Two of the patients had onset of severe generalized cystic acne concomitant with their development of PRP. PRP and acne conglobata should be added to the group of cutaneous disorders that can present in a more virulent manner in the setting of HIV infection. The association of cystic acne with PRP and their response to treatment are discussed.