Chronic myeloid leukemia (CML) is a chronic myeloproliferative disorder that usually presents with high white blood cell counts and massive splenomegaly. Priapism is a rare manifestation of CML and is mostly due to hyperleukocytosis. Its debut appearance as a sign of hematological dyscrasia is a rare event. Priapism occurring in a setting of any leukemia is both a medical and a urological emergency that requires immediate local therapy, symptomatic treatment, cytoreductive therapy and early initiation of targeted therapy. This case report describes priapism as an unusual presentation of CML and its importance in the work-up and management of patients presenting with priapism.