We describe a 62-year-old patient with a 4-year history of myelodysplasia who later developed striking features that included massive splenomegaly, rapidly evolving visual loss and a sensorimotor polyneuropathy. This led us to consider the diagnosis of haemophagocytic lymphohistiocytosis (HLH). Upon further investigation, we found that he fulfilled the necessary diagnostic criteria for HLH, including the presence of haemophagocytosis of erythroid precursors on bone marrow smear.