Sickle cell/beta (0)-thalassemia (S/β(0)-thal) is a compound heterozygous state for βS and β(0) thalassemia. There are rare reported cases of patients with sickle cell disease who developed hematological neoplasms including myeloid and lymphoid conditions; however, to the best of our knowledge, chronic myelogenous leukemia (CML) occurring in S/β(0) -thal has been reported in one case and this is the second such report.