We present a 66-year-old male patient with pulmonary lymphomatoid granulomatosis. The patient had progressive disease after three courses of CHoP and rituximab and, therefore, treatment with interferon-&αlpha;2b 5 x 10<sup>6</sup> iE three times a week was started. This resulted in stable disease for five months. subsequently, progression occurred and the patient died 12 months after initial presentation. lymphomatoid granulomatosis is a rare, poor-risk, Epstein-Barr virus related, B cell lymphoproliferative disease. There is no standard treatment but promising results have been reported with rituximab, either as monotherapy or in combination with chemotherapy. This case demonstrates that lymphomatoid granulomatosis is still a chemotherapyresistant disease in some patients despite addition of rituximab. A review of the literature regarding aetiology, clinical features, diagnosis and treatment options is presented.