Chronic myeloid leukemia (CML) is a type of myeloproliferative neoplasm (MPN) in which granulocytes are the primary proliferating cells. CML in pediatric patients (juvenile CML) is an uncommon MPN, representing 2-3% of children newly diagnosed with leukemia. Sensorineural hearing loss that progresses rapidly is an uncommon early sign of a hematological disorder in patients with CML. This article presents the case of a 10-year-old patient with complaints of bilateral hearing loss for 2 weeks. Pure-tone audiometry indicated sensorineural hearing loss of the left ear and mixed hearing loss of the right ear. After an in-depth evaluation of a bone marrow smear and positivity for the BCR-ABL fusion gene, it was concluded that the patient had chronic-phase juvenile CML. However, hearing loss recovery after hydroxyurea therapy could not be observed because the patient died after suffering an uncontrolled seizure on day 14 of hospitalization.