Myeloid sarcoma is characterized by the presence of myeloid blasts at an extramedullar site that disrupts the normal architecture of the organ. Many of these cases are associated with acute myeloid leukemia, chronic myeloid leukemia, myelodysplastic syndrome, or occur in de novo. It occurs most commonly in skin, lymph node, gastrointestinal tract, bone, soft tissue but, rarely in head and neck; especially in nasal cavity. Therefore, it is often misdiagnosed as a different disease: most commonly as lymphoma. Here we report a rare case of myeloid sarcoma in the nasal cavity that occurred in a patient with leukemic transformation with myelodysplastic syndrome, provided with literature review.