Background: Bone marrow necrosis, a rare condition, characterized by necrosis of the medullary stroma and myeloid tissues of the bone marrow. The etiology is diverse, including hematopoietic malignancies.Case report: A 54-year-old male arrived by ambulance with collapse and high fever. On clinical examination, he had numerous petechiae and ecchymoses in the mouth and on the limbs. Laboratory findings revealed anemia and thrombocytopenia (WBC 15,140/μl, Hb 4.7g/dl, Plt 3.8X104/μl). A blood culture was positive for Staphylococcus aureus, diagnosed as septic shock, complicated by disseminated intravascular coagulation (DIC). When his medical condition was improved after treatment, blood examination showed pancytopenia (WBC 2,170/μl, blast 1.5%, Hb 9.2g/dl, Plt 1.1X104/μl). Multiple bone marrow aspiration displayed only small amount of the cells, which were difficult to be classified and were negative for peroxidase. Bone marrow biopsy showed marked bone marrow necrosis. During being treated for DIC, the abnormal cells were appeared in the peripheral blood, which were analyzed by flow cytometry and cytogenetics and eventually he was diagnosed as AML (M0, FAB classification). The sudden appearance of consolidation in the lungs by chest X-ray indicated leukemic cells infiltration into the lungs. His physical status was allowed chemotherapy consisting of cytarabine and aclarubicin only for the first four days of the standard protocol. Laboratory findings were improved, however, he suffered from repeated hemoptysis. Thoracic CT scan demonstrated diffuse alveolar damage and possible pneumonia. Despite of supportive treatment, his condition was complicated with chemotherapy associated-febrile neutropenia and he expired four weeks after admission.Conclusion: Bone marrow necrosis prognosis is related to underlying diseases and is generally poor. Therefore, multiple bone marrow aspirations and biopsies are needed to achieve appropriate diagnosis.