Lymphoblastic lymphoma is a rare disease in adults, primarily affecting patients in their late teens and early 20s. Optimal treatment strategies have been slow to emerge because of the rarity of this disease and the variable distinction in the clinical literature between this condition and acute lymphoblastic leukemia. Although these two conditions are now regarded as a single entity in the WHO Classification of Lymphoid Neoplasms, treatment approaches have developed separately, and recent molecular data suggest that there may be important biologic differences between these conditions that may justify a different treatment approach. Most published data support the use of intensive multiagent chemotherapy induction followed by a consolidation and maintenance phase. Optimal consolidation remains unclear, although there is no clear role of stem cell transplantation after intensive remission induction therapy based on current evidence. Emerging molecular data have identified potential new therapeutic targets with supporting preclinical data.