The development of Hodgkin's lymphoma (HL) is a known complication in patients with human immunodeficiency virus (HIV) infection. Extranodal involvement, specifically primary bone marrow Hodgkin's lymphoma (PBMHL) is a rare manifestation that has been reported in HIV-positive patients and may represent a distinct entity from HIV-associated HL. We present a case of PBMHL presenting with hemophagocytic lymphohistiocytosis (HLH) in an HIV-positive patient. The 55-year-old male with HIV/AIDS presented with abdominal pain, diarrhea, and fever, leading to hospital admission. Despite initial treatment, he deteriorated, prompting re-admission and investigation revealing pancytopenia and elevated inflammatory markers, suggestive of HLH. Subsequent bone marrow biopsy unexpectedly revealed PBMHL. Treatment with HLH-directed therapy and the HLH-94 protocol resulted in significant clinical improvement. This case underscores the importance of recognizing atypical lymphoproliferative presentations in HIV/AIDS patients and the need for interdisciplinary collaboration in complex cases.