Hemochromatosis is associated with increased risk of hematological neoplasias, but studies showing hemochromatosis gene mutations in myelodysplastic syndrome (MDS) are scanty, particularly in the elderly. The onset of MDS in hemochromatosis usually occurs between 60 and 70 years of age, while cases with advanced age are very rare. We report a case of a 78- year-old man with hemochromatosis who developed refractory anemia with excess of blasts. Our case suggests that in the elderly with hemochromatosis, myelodysplasia should be considered a possible cause of anemia.