Systemic sclerosis is a chronic multisystem disorder of unknown etiology characterized by the involvement of skin and visceral organs caused by an accumulation of collagen. It has been reported that the incidence of solid and hematological malignancy increased in systemic sclerosis. Multiple myeloma and chronic lymphocytic leukemia are the most common hematological malignancies seen in patients with systemic sclerosis. Chronic myeloid leukemia (CML) has only rarely been reported so far. We here report a case with CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactly, telangiectasia) who developed CML 7 years after the onset of CREST. Ours is the second case with CML developing after the onset of CREST in the literature. We also briefly discuss the possible tendency to hematological malignancy in systemic sclerosis.