Approximately 5% of patients present with two different histologic subtypes of lymphoma at the time of diagnosis, usually with low grade lymphoma in the bone marrow or a lymph node and large cell lymphoma at another lymph node or extra-nodal site. Initial management should be as appropriate for the most aggressive subtype present, the apparent extent (stage) of the aggressive subtype and the age of the patient. Subsequent relapse is common, but may occur after a long interval and may be from either of the presenting types of lymphoma. Treatment of the relapse depends on the histologic type and clinical behaviour of the disease.