We conducted a retrospective review of pathology files and hospital records and identified three unusual presentations of granulocytic sarcoma associated with acute myeloid leukemia (AML) of the head and neck. At least one mass was observed on the skin of all three patients. A 17-year-old boy had masses in each temporal region that were accompanied by bilateral facial paralysis. He was administered chemotherapy and radiotherapy, but he died of infection secondary to a second relapse 29 months after the initial diagnosis. A 17-year-old girl had a tumor in the right parotid area. She received chemotherapy, but she died of infection and bleeding 2 months after the initial diagnosis. A 33-year-old man had numerous tumors widely disseminated over his skin. He received chemotherapy and was in remission 12 months after the initial diagnosis, but he eventually relapsed and died. Granulocytic sarcoma can be localized in unexpected regions, including the head and neck. This tumor is very often misdiagnosed as a malignant lymphoma, which leads to delayed treatment and a poor outcome. Therefore, clinical and histopathologic findings should be evaluated before any diagnosis of malignant lymphoma is pronounced. Immunohistochemical stains should also be performed on patients with suspected granulocytic sarcoma, and aggressive chemotherapy or immunotherapy should be administered. We believe that high-dose chemotherapy can improve survival rates in granulocytic sarcoma associated with AML.