Myeloid sarcoma (MS) is a rare, solid malignant tumor formed by the proliferation and infiltration of myeloid cells or immature myeloid cells in the extramedullary tissue. MS is usually associated with acute myeloid leukemia (AML). AML is a malignant disease of myeloid hematopoietic stem cells. Most cases progress rapidly and the prognosis is dangerous. MS can occur anywhere in the body, but it is even rarer when it occurs in the nasal sinuses. This paper reports a case of sinusoidal MS complicated with AML in Tsinghua Changgeng Hospital of Tsinghua University in Beijing. The clinical features, diagnosis, treatment and prognosis of sinusoidal MS are reviewed.