Acquired immune deficiency syndrome (AIDS)-related lymphomas (ARL) still represent a relevant field of clinical research. For most histological subtypes of ARL, no optimal initial therapy has been clearly defined so far. Rituximab plus chemotherapy is feasible and effective and should be offered to all patients with CD20-positive ARL regardless of their CD4 cell count. Combination antiretroviral therapy (cART) should be given concomitantly with chemotherapy, bearing in mind potential drug-drug interactions. Appropriate treatment of ARL is determined by a number of factors such as lymphoma stage, performance status, comorbidities, histological subtype, and immunosuppression. Treatment should principally be the same as in human immunodeficiency virus (HIV)-negative lymphoma patients. In HIV-related Hodgkin's lymphoma, high cure rates have been achieved with stage-adapted treatment approaches, albeit with worse outcomes compared to immunocompetent patients.