Adult T-cell lymphoblastic lymphoma is a relatively rare aggressive type of non-Hodgkin lymphoma with frequent involvement of extranodal sites. Because of the rarity of this malignancy, it is treated variably and often suboptimally, using approaches similar to those used for other types of aggressive non-Hodgkin lymphomas, with the consequence that outcome is often suboptimal. The collective experience in the management of adult T-cell lymphoblastic lymphoma suggests a good outcome for patients with no adverse prognostic factors who are treated with an acute lymphocytic leukemia-like treatment strategy. Patients with adverse prognostic features should be considered for more aggressive therapy-specifically, high-dose chemotherapy and hematopoietic stem cell transplantation. This article will attempt to review the current status of chemotherapy treatment programs and the relative merits of the different hematopoietic stem cell transplantation programs in this disease, particularly in relation to the pathologic and clinical features that correlate with disease prognosis.