Adult cell lymphoma/leukemia (ATLL) is a type of lymphoma consisting of T-cells that are related to infection with the human T lymphotropic virus (HTLV-1). Four clinical forms have been described (leukemic, lymphomatous, chronic, smoldering) and the phenotype corresponds to regulatory CD4+ T cells. The histological characteristics are variable, with neoplastic cells showing a size ranging from small to large and atypical nuclei with irregular contours. A series of genetic and molecular alterations have been described, which partially explain the lymphomagenesis of the neoplasm, some of which are also factors related to the clinical course and overall survival. ATLL is a neoplasm with a poor prognosis, but in recent years new targeted therapies have been designed, with encouraging responses. This neoplasm should continue to be studied to improve treatment and evolution.