Chronic myeloid leukemia (CML) is a cancer of the white blood cells and the bone marrow. It is characterized by an increased and unregulated growth of myeloid cells in the bone marrow and accumulation of these cells in the blood. There are three stages of CML, and most patients are diagnosed incidentally with an elevated white blood cell count seen on the complete blood count (CBC). Occasionally, CML patients present with upper quadrant pain due to hepatosplenomegaly. An enlarged spleen may put pressure on the stomach, causing loss of appetite and weight loss. CML patients may also present with fever, night sweats, bleeding, or petechiae. An initial presentation of the CML patient with priapism is very unusual and rare. In this case report, we are documenting a case of a 22-year-old man who presented with a persistent painful erection of the penis for four days. After emergency treatment for the priapism, the patient underwent multiple investigations including blood morphology, bone marrow aspiration, and BCR-ABL molecular testing, which confirmed the diagnosis of CML. The purpose of this study is to highlight the rare presentation of CML and the importance of diagnosing the underlying cause responsible for the acute presentation.