Hemophagocytic lymphohistiocytosis (HLH) is a rare and deadly disease that presents significant diagnostic challenges. The complexity of diagnosis and treatment is further increased among transplant recipients, yet reports on solid organ transplants, and specifically heart transplant recipients, remain scarce. Herein, we elucidate the diagnostic journey, clinical evolution, and therapeutic approach undertaken for a heart transplant recipient afflicted with hemophagocytic lymphohistiocytosis, followed by a review of the literature.