Introduction: BCR-ABL positive acute myeloid leukaemia is recognised as a provisional entity in the WHO 2016 classification for AML and related precursor neoplasms. Myeloid sarcoma is an extramedullary accumulation of immature myeloid cells, intracranial occurrence is rare.

Case report: A 40-year old male presented with an intracranial mass and peripheral blood blastosis of 65%. A diagnosis of acute myeloid leukaemia with intracranial myeloid sarcoma was presumed, requiring urgent cranial decompression. The patient received standard AML induction therapy with daunorubicine and cytarabine. Molecular analysis surprisingly revealed BCR-ABL positivity.

Discussion: BCR-ABL positive AML is a rare entity and needs to be differentiated from CML in blast crisis because of therapeutic consequences. In our case, clinical and molecular features suggested a diagnosis of AML.

Conclusion: To the best of our knowledge, this is the first case of intracranial myeloid sarcoma as a concurrent presentation of BCR-ABL-positive acute myeloid leukaemia.

(BELG J HEMATOL 2021;12(4):165-8)