Myeloid sarcoma is a rare tumor of extramedullary immature myeloid cells and very few cases of nasopharyngeal involvement have been documented. Despite being most common in skin, bone and lymph nodes; it can be seen in any region with nasopharynx being one of the rare sites. In this article, we aimed to present a case of nasopharyngeal myeloid sarcoma. 58 -year -old male who was previously diagnosed with AML transformed from MDS and treated with chemotherapy, presented with complaints of hearing loss in the right ear and swelling in the right side of the neck. He had a mass lesion in the right half of the nasopharynx in the endoscopic examination performed upon the presence of hearing loss and serous otitis findings. Imaging findings revealed a mass lesion starting from the right half of the nasopharynx extending to the posterior of the carotid space and paravertebral area. Patient was diagnosed with acute myeloid leukemia (AML) transformed from myelodysplastic syndrome (MDS) and was treated with chemotherapy regime consisting of cisplatin and concomitant radiotherapy. Since myeloid sarcoma is a rare tumor seen in 3-8% of patients with AML, high suspicion and immunohistochemical analysis are important in the diagnosis of this tumor.