An 8-year-old boy with a 3-day history of tense, rigid, and painful penis presented to the urology department. There was no history of trauma or medication use. The physical examination showed splenomegaly and laboratory investigations revealed leukocytosis with left shift, suggesting the possibility of a myeloproliferative neoplasm. The patient underwent corporal-glandular shunt surgery for priapism, successfully resolving the condition. However, the further evaluation indicated pediatric chronic myeloid leukemia (CML). Treatment with Dasatinib achieved a complete hematologic response within one month. This case is a reporting of rare phenomena of CML and priapism as a presenting feature in a pediatric patient and a comment on the treatment challenges of Pediatric CML.