Background: Chronic Myeloid Leukemia (CML) is a rare malignancy among children, having an incidence of 1 in a million. It is driven by the Philadelphia chromosome, resulting in the BCR-ABL1 fusion gene. Pediatric cases typically present in the chronic phase and are managed with tyrosine kinase inhibitors (TKIs) like imatinib. However, some cases progress to blast crisis, leading to life-threatening complications such as acute respiratory distress syndrome (ARDS) as in our case. Methods: We present a case report with clinical and laboratory data, along with a brief literature review. Case Presentation: We present the case of a 14-year-old female diagnosed with adult-type CML, confirmed by the presence of the Philadelphia chromosome. Initially she was managed with imatinib and remained stable for one year before presenting with blast crisis complicated with acute respiratory distress syndrome (ARDS). Laboratory findings revealed a white blood cell count of 31.3 x10 9/L and a blast count exceeding 20%. A CT scan showed hepatosplenomegaly, and the patient was treated in the ICU with antibiotics, oxygen therapy, and blood transfusions. Despite the severity of the blast crisis, the patient’s condition stabilized after three weeks of intensive care and was discharged for follow-up and resumption of imatinib. Conclusion: This case highlights the aggressive nature of pediatric CML and the potential for life-threatening complications such as ARDS during the blast crisis phase. Early detection and intervention are crucial for favorable outcomes. Although TKIs are the primary treatment for pediatric CML, hematopoietic stem cell transplantation remains the only curative option for advanced stages or refractory cases.