Human immunodeficiency virus (HIV)-infected patients carry an increased risk of lymphomagenesis. Although the majority of HIV-related lymphomas have a B-cell phenotype, the incidence of peripheral T-cell lymphomas (PTCL), including primary cutaneous subtypes, may be up to 15-fold higher than in the general population, with anaplastic large cell lymphomas (ALCL) accounting for 18–28% of HIV-associated PTCL. In contrast to systemic ALCL, the relation between HIV infection and primary cutaneous ALCL has been relatively neglected in the literature. We report the case of a primary cutaneous ALCL occurring in a 76-year-old patient with advanced HIV infection, and showing unusually aggressive course. Neither ALK1 immunohistochemical positivity nor evidence of EBV infection were detected; staging procedures at initial presentation ruled out systemic involvement. We provide a summary of the literature regarding primary cutaneous ALCL in HIV-infected patients. We draw attention to clinicopathological features, prognostic implications and therapeutic quandaries of HIV-related primary cutaneous ALCL. Further, we propose that a significant fraction of HIV-associated cases might represent a more aggressive subset of primary cutaneous ALCL.