Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) or lymphogranulomatosis X is a lymphoproliferative disorder with a histological picture resembling that of reactive lesions but with frequent cytogenetic and molecular abnormalities characteristic of malignant T cell lymphoma. Clinically, the disease runs a fatal course in the majority of patients although occasional spontaneous remissions have been observed. Median survival approaches only 1 year even with the most effective treatment protocols implemented so far. Fewer than 20% of patients survive 5 years after diagnosis and cure seems exceedingly rare. High-dose chemotherapy (HDCT) followed by autologous bone marrow transplantation (ABMT) represents a promising new treatment modality for patients with advanced lymphoma conceivably including AILD. We report the first patient with relapsed AILD successfully treated by HDCT and ABMT. This 21-year-old male is alive and free of disease 27 months after ABMT with a Karnofsky score of 100%.