This paper highlights a case of haemophagocytic lymphohistiocytosis (HLH), a rare but important complication of checkpoint inhibitor (CI) therapy which are widely used in the treatment of solid organ and haematological malignancies. We present a case report, diagnostic work-up, associated challenges in management and review the existing literature regarding HLH secondary to CI therapy. Our findings suggest that treatment discontinuation and high-dose corticosteroid therapy without chemotherapy may be sufficient in treating HLH related to CI therapy.