Background: Chronic myeloid leukemia (CML) is a hematological malignancy caused by the expression of the aberrant oncogene “BCR-ABL” attributed to t (9:22) Philadelphia translocation. The coexistence of pregnancy and CML is rare; less than 1 in 100,000. Managing a case of CML in pregnancy remains a clinical dilemma due to serious ethical and therapeutic implications which chemotherapy during pregnancy can pose for the fetus like various congenital malformations, abortion, and intrauterine growth restriction. Case description: We present a case report of a 28-year-old female at 34.2 weeks of gestation with previous 2 lower segment cesarean sections in preterm labor. The patient was a known case of CML on Imatinib mesylate, who opted to discontinue chemotherapy in view of her ongoing pregnancy. She subsequently presented in labor in accelerated phase of CML, further complicated by placental abruption. Following an emergency cesarean section and ensuing disseminated intravascular coagulation (DIC), she ultimately succumbed to tumor lysis syndrome despite multidisciplinary critical care management. Conclusion: The treatment of pregnant women with CML is challenging due to limited therapeutic options with sparse data regarding safety in pregnancy. Conception should be planned after achieving major molecular response and continuation of pregnancy and chemotherapy needs to be individualized after detailed counseling involving the obstetrician and the hemato-oncologist. Our case report, hereby, aims to emphasize the importance of preconceptional counseling, shared decision-making, and rigorous follow-up during pregnancy.