ObjectiveTo discuss the clinical features of primary extra nodal lymphoma in head and neck region and to summarize the causes of misdiagnosis.MethodsClinical data of 146 patients with pathologically confirmed primary extra nodal lymphoma in head and neck was analyzed retrospectively.ResultsOf all the 146 patients, 86 were male and 60 were female. As for the primary lesions, the lymphoma located in nasopharynx in 50 cases, tonsil in 42, thyroid gland in 33, nasal cavity in 11, larynx in 3, major salivary glands in 2 cases, root of tongue in 2, hard palate in one, scalp in one, and buccal mucosa in one.ConclusionsPrimary extra nodal lymphoma may occur at multiple sites of head and neck. This disease is usually misdiagnosed due to its hidden primary lesion, nonspecific clinical symptoms, and clinicians’ lack of awareness. To improve the diagnosis rate and to avoid inappropriate treatment, the suspected patients should be followed up strictly, timely (aspiration) biopsy and intraoperative rapid pathologic examination should be performed if necessary.