Discrete diagnostic subtypes of T lymphoblastic leukemia/lymphoma (T-cell acute lymphoblastic leukemia/lymphoma, T-ALL) have historically not been widely recognized. Recently, a novel subset with distinctive immunophenotypic, molecular, and clinical features has been proposed. Termed early T-cell precursor acute lymphoblastic leukemia (ETP-ALL), these cases seem to correspond to a very early stage of T-cell development. ETP-ALL is associated with a poor prognosis using standard protocols, and patients with ETP-ALL may benefit from intensified, alternative, or targeted therapies. Recognizing ETP-ALL and distinguishing it from other forms of acute leukemia are important elements of an up-to-date diagnostic approach to precursor T-cell neoplasms.