Due to the lack of therapeutic options for patients with progressive multifocal leukoencephalopathy-associated immune reconstitution inflammatory syndrome (PML-associated IRIS), maraviroc has generated expectations among the medical community. However, we report a patient with advanced HIV infection, who developed PML-associated IRIS and had a fatal outcome despite the addition of maraviroc to suppressive ART. Future studies are required to define the therapeutic role of maraviroc in PML-associated IRIS and differentiate individuals who may benefit from maraviroc from those who may develop neurological deterioration.