Haematological involvement of systemic lupus erythematosus (SLE) - which ranges from the well-described haemolytic anaemia to macrophage activation syndrome - has a large impact on both morbidity and mortality. On the other hand, association between haematological malignities and SLE - in terms of pathophysiology and molecular genetics - is an obscure entity which has not been clarified evidently to date. Herein, we present a six-year-old female with the diagnosis of SLE who developed acute lymphoblastic leukaemia following a period of myelodysplasia. It could possibly be coincidental; however, persistent cytopenia, prominent dysplasia on bone marrow smears and azathioprine treatment may be considered as possible triggers for the development of leukaemia in the present case.