Granulocytic sarcoma (GS) is a rare localised tumour of malignant myeloid precursor cells occurring at an extramedullary site. It is usually associated with a myeloproliferative disorder but may be seen preceding the onset of leukemia. Extracranial GS may occur virtually anywhere in the body and may be easily confused with large cell and lymphoblastic lymphoma. This paper reports an unusual case of primary GS of the nasal cavity and paranasal sinuses, which presented as a nasal obstruction. Multidrug combination chemotherapies and bone marrow transplantation were performed. Awareness of the potential location of GS in the nasal cavity and paranasal sinuses should contribute to better define the prognostic significance of this uncommon entity.