BACKGROUND: T cell large granular lymphocytic leukemia (T-LGL) is a rare indolent lymphoproliferative disorder caused by aberrantly clonal expansion of cytotoxic T lymphocytes. Active mutations of CASE DESCRIPTION: A 36-year-old female had a history of T-LGL and multiple autoimmune disorders, including type I diabetes mellitus, pure red cell aplasia, IgA deficiency, celiac disease and recurrent infection and bacteremia. She carries an HLA-DQ8 allele and a germline heterozygous mutation of CONCLUSIONS: We report an extremely rare case of autoimmune enteropathy associated T-LGL in a patient with non-