Abstract: The patient was admitted with left-sided headache accompanied by rhinocleisis that persisted for a month. The patient was diagnosed with adenoid cystic carcinoma of the nasopharynx. Six months previously, the patient had been treated with standard radiotherapy (DT: 200cGy, 17 times) and gamma knife (35.6Gy), but the tumor recurred, prompting this admission. After admission, the patient was given systematic chemotherapy with DDP+THP+FUDR for a week, but the symptoms were not relieved. Local gamma knife was applied (400cGy, 5 times) and the pain was slightly relieved. We discuss the pathology, image features, surgery, radiotherapy and chemotherapy of this disease and establish a standard treatment plan. Nasopharynx primary adenoid cystic carcinoma is rare. It appears as an invasive growth and mostly occurs in women. Due to the complicated structure of the nasopharynx and the cancer's tendency to invade vessels and nerves, radiotherapy is preferred over surgery. The 5-year local control rate and survival rate are 21% and 31%, respectively.