We report a case of a 26-year-old Caucasian male presented with 8-month history of asymptomatic erythematous papulonodular lesions symmetrically located on the face, trunk and limbs. Histopathologic examination revealed granulomas with histiocytes arranged in a palisading pattern around the foci of necrobiotic collagen with mucin deposition, and transepidermal and transfollicular elimination of degenerated collagen bundles. These histopathologic findings were compatible with perforating granuloma annulare. Three months later, the patient was admitted with the diagnosis of sepsis secondary to a gastroenteritis dyeing on the fourth day of presentation. Serologic tests for antibody to human immunodeficiency virus (HIV) performed at this time were positive establishing the diagnosis of acquired immunodeficiency syndrome (AIDS). Atypical variants of granuloma annulare including perforating types might be associated with AIDS. The course and presentation of granuloma annulare might be modified by disruption of both antigen presentation and effector cell mechanisms in the HIV-infected patient.