We describe a patient with trilineage myelodysplasia and hyperplastic megakaryopoiesis associated with significant myelofibrosis at diagnosis, who developed acute myeloid leukaemia two months afterwards. The clinical and pathological features were consistent with a myelodysplasia/myelofibrosis syndrome with leukemic transformation. Cytogenetic investigation showed trisomy 14, del(20q) and t(3;3) (q21;q26). These findings are of possible significance in the understanding of the association between myelodysplasia and myelofibrosis.