Acromegaly is an uncommon disease characterized clinically by enlargement of the acral parts, coarse facial features and visceralomegaly. In most patients, the disorder arises from a tumor of the anterior pituitary gland that secretes excessive amounts of growth hormone. A few cases have been reported, associated with the development of leukemia due to increased growth hormone. But, chronic myelogenous leukemia has not been reported in patients with acromegaly. We report a case of chronic myelogenous leukemia in patients with situs inversus and acromegaly. A 70-year old male patient who was admitted to endocrinology department because of acromegalic features was referred to the hematology department for the abnormal hematologic findings. LAP score was decreased. Bone marrow aspiration and biopsy revealed chronic myelogenous leukemia. Chest radiographs showed a dextrocardia. Abdominal sonography showed a splenomegaly and transposition of liver and spleen. Growth hormone level was elevated and pituitary tumor was detected on brain MRI.