Acute lymphoblastic leukemia (ALL), also known as acute lymphoid leukemia or cancer of the white blood cells, develops from lymphocytes overproduced in the bone marrow that spread to other sites. Survival in ALL has improved in recent decades, owing mostly to combination chemotherapy, preventive CNS-directed therapy, improved risk stratification based on cytogenic features, and early response. In the last few years, several agents have launched that may improve treatment outcomes for patients with ALL, including monoclonal antibodies to CD19 (blinatumomab, Amgen’s Blincyto) and CD20 (Roche/Genentech’s rituximab, [Rituxan]); new drugs for T-cell ALL, such as nelarabine (Novartis’s Arranon/Atriance); and the TKI agents imatinib (Novartis’s Gleevec/Glivec), dasatinib (Bristol-Myers Squibb’s Sprycel), and ponatinib (Ariad Pharmaceuticals’ Iclusig) for the treatment of Ph+ ALL. Nonetheless, chemotherapy remains the backbone of first-line ALL treatment. Therapies in development include immunomodulatory agents, which hold great promise of fulfilling the need for more-effective therapies for this indication.