We present a case of Hodgkin's disease of the mastoid associated with upper cervical lymph node involvement without disseminated disease. This patient was treated with chemotherapy alone and lesions responded rapidly. At present the patient is well, with no evidence of recurrence or extension of this disease. Hodgkin's disease of the middle ear and mastoid is an extremely rare disease. After a wide search in the literature no cases of Hodgkin's disease arising in the middle ear and mastoid have been found.