Enteropathy-associated T cell lymphoma(EATL)is a rare subtype of mature T cell lymphoma,formerly known as EATL type Ⅰ.EATL is closely linked to celiac disease.Risk factors for EATL include a history of celiac disease,human leukocyte antigen-DQ2(HLA-DQ2)gene susceptibility,aberrant intestinal intraepithelial lymphocytes(IELs)accumulation and ulcerative jejunitis.The pathogenesis is related to HLA-DQ2 allele variation,mutational events of Janus kinase(JAK)-signal transducer and activator of transcription(STAT)pathway and nuclear factor kappa-B(NF-κ B),as well as functional changes of microRNA(miRNA).Commonly involved sites in EATL are the small intestine,followed by the stomach and colon.EATL usually presents with dyspepsia,gluten-insensitive malabsorption,intestinal obstruction,hemorrhage and perforation.EATL is an aggressive disease,with a poor prognosis.Misdiagnosis is common during the early stage of the disease.Anthracycline-based combination chemotherapy is the dominant treatment for EATL.Surgical resection is used to alleviate obstruction,hemorrhage,or perforation in some patients.Stem cell transplantation for consolidation after remission of first-line chemotherapy can prolong patients'survival time.Monoclonal antibody brentuximab vedotin targeting CD30,anti-CD52 antibody alemtuzumab,immune checkpoint inhibitors,and drugs used to treat refractory celiac disease(RCD)type Ⅱ and peripheral T cell lymphomas(PTCL)may be effective against EATL.This paper summarizes the epidemiology,genetic and molecular characteristics,clinicopathological characteristics,immunophenotypic characteristics and new advances in the treatment of EATL.