Systemic sclerosis is uncommonly associated with hematologic malignancies. We report the case of a patient who had chronic myelogenous leukemia 3 years after the CREST variant of systemic sclerosis was diagnosed. She also later had porphyria cutanea tarda. The majority of patients who had hematologic malignancies after the diagnosis of systemic sclerosis proved to have either multiple myeloma or chronic lymphocytic leukemia. Hematologic malignancies may be found in patients with systemic sclerosis (either limited or diffuse).