Type II enteropathy-associated T-cell lymphoma (EATL) is a rare disease that is reported from Asia, especially from Taiwan. In this paper, we present a patient from Iran (Arab) with Type II EATL whose disease was not associated with celiac disease or Epstein-Barr virus (EBV) infection. The patient died due to intestinal perforation during the first chemotherapy. Type II EATL is a gastrointestinal lymphoma with poor prognosis and a high affinity for intestinal perforation. Early detection of EATL II is essential for chemotherapy prior to the onset of malnutrition symptoms.