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The authors report a case of endobronchial non-Hodgkin lymphoma, in a 48-year-old woman. This is an exceptional localization. When it reveals the disease, as was the case in the index patient, clinical presentation is usually atelectasis with fever. Diagnosis was established upon bronchoscopy. Histologic examination of biopsy specimens showed an immunoblastic lymphosarcoma. Total pneumonectomy and chemotherapy failed to prevent rapid deterioration with a fatal outcome 7 months after onset. A selective total IgA deficiency and a familial history of malignant tumors were recorded. Immunoblastic sarcoma has a poor prognosis. It often follows a disease whose mechanism is ascribed to an immunological disorder. Lymphoma arising in a patients with prior immune disease should suggest the diagnosis of immunoblastic sarcoma.