Introduction: severe coagulation disorder is a life-threatening complication, usually occurring in acute promyelocytic leukemia (APL), however it is rare in all other subtypes of acute myelogenous leukemia (AML). A typical feature of disseminated intravascular coagulation (DIC) is hyperfibrinolysis and reduction of protein C and antithrombin III. The leukemia cells release tissue factor (TF), that induces endothelial cells disruption leading to enhanced TF activation in vessel walls or the increase of inflammatory cytokines that upregulate TF activity in the endothelium or monocytes. Increased TF release activates the extrinsic coagulation pathway, that added to insufficient anticoagulation rescue by activated protein C, results in DIC in leukemia patients, both at the time of diagnosis and during cytotoxic chemotherapy, due the tumor lysis. This clinical condition must be identified and treated immediately, due to its severity. This report illustrates a case of severe DIC with central nervous system (CNS) bleeding in a pediatric patient with acute monoblastic leukemia. Case report: a 16 year-old female was admitted in our service with fatigue and back pain and pancytopenia (Hb = 5 g/dL. WBC = 1.950/mL with 5% blast cells, platelet count = 43.000/mL). She had no fever, lymphnodes, liver or spleen enlargement nor bleeding signals. A bone marrow smear detected 70% myeloid blasts, and the immunophenotype revealed AML with monoblastic differentiation (cyMPO+dim, CD4+dim, CD13+, CD38+, CD45+intermediate, CD117+dim/negative, CD123+, HLA-DR+, and bright positive CD15, CD33, CD56 and CD64). FISH did not detect PML-RARA fusion. Hematological karyotype detected translocation t(8;16). During the second day of hospitalization, the patient developed severe headache, drowsiness and vomiting. A cranial computer tomography evidenced extensive right frontal cerebral hematoma. Laboratory testing revealed prolonged PT-INR = 3,21 and APTT = 77sec, and Fibrinogen = 1,2 g/dL (normal: 1,5 – 3,5 g/L). The patient was transferred to the intensive care unit and transfusion support with fresh frozen plasma, thrombocyte and erythrocyte concentrates was initiated. On the third day, chemotherapy was started based on AML-BFM 2008, without Idarubicin, and support for tumor lysis syndrome. However, she presented neurological impairment, with anisocoria and respiratory failure requiring mechanical ventilation. Emergency craniotomy was successfully performed for intracranial decompression. Despite the intensive therapy, the patient maintained DIC and developed renal failure and death by sepsis in febrile neutropenia (germs have not been isolated) and pulmonary alveolar bleeding on the 29th day of hospitalization. Discussion and conclusion: the control of bleeding is essential during the induction therapy of AML patients. Chemotherapy can improve the DIC outcome by controlling leukemia, always associated with sources of fibrinogen replacement and agents that convert protein C into the activated form, not widely available in our country. Although the incidence of DIC is rare in non-APL AML, this knowledge is important, which allows the immediate recognition and treatment of this condition, allowing a better outcome for these patients.