Enteropathy-associated T-cell lymphoma(EATL) is a rare peripheral T-cell lymphoma classified into 2 types,with or without celiac disease,based on histology.Type 2 EATL is less commonly associated with celiac disease,in which cells are characterized by being monomorphic and small-to medium-sized.Cells are characterized by CD8 and CD56 expression and c-MYC oncogene locus gain.We present an atypical case of type 2 EATL in the jejunum,with human T-lymphotropic virus-1 that was CD4-CD8+ CD56-CD30-CD25-TIA-1+ and granzyme B+ on immunohistological staining.It also displayed translocation of chromosome 8p24(c-MYC),as determined by fluorescent in situ hybridization.Mucosal spreading and intraepithelial invasion by lymphoma with villous atrophy were detected adjacent to the mucosal layer.The lymphoma may be derived from intraepithelial CD8+ T cells,similar to celiac disease. Enteropathy-associated T-cell lymphoma (EATL) is a rare peripheral T-cell lymphoma classified into 2 types, with or without celiac disease, based on histology. Type 2 EATL is less commonly associated with celiac disease, in which cells are characterized by are monomorphic and small-to-medium-sized. Cells are characterized by CD8 and CD56 expression and c-MYC oncogene locus gain. We present an atypical case of type 2 EATL in the jejunum, with human T-lymphotropic virus-1 that was CD4 -CD8 + CD56-CD30-CD25-TIA-1 + and granzyme B + on immunohistological staining. It also displayed translocation of chromosome 8p24 (c-MYC), determined by fluorescent in situ hybridization. Mucosal spreading and intraepithelial invasion by lymphoma with villous atrophy were detected adjacent to the mucosal layer. lymphoma may be derived from intraepithelial CD8 + T cells, similar to celiac disease.