ALK negative anaplastic large cell lymphoma (ALK(-) ALCL) lacks the specific expression of ALK protein, although it also strongly expresses CD30 and resembles the morphologic characteristics of ALK positive anaplastic large cell lymphoma (ALK(+) ALCL). Recently, some new researches indicate that there exist molecular and genetic differences between these two types of ALCL. Moreover, the treatment response, prognosis, and long-term survival of ALK(-) ALCL are far worse than that of ALK(+) ALCL, such as ALK(-) ALCL is associated with older age persons, B group syndrome, disease advanced stage, high International Prognostic Index (IPI) and poor prognosis (< 49% 5-year survival). As a consequence, some new advances on basis (cell morphology and tissue pathology, immunophenotypes, cell genetics and molecular marker), diagnosis and treatment of ALK(-) ALCL are summarized in this review.