Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare primary and highly aggressive intestinal T-cell lymphoma derived from intraepithelial lymphocytes. MEITL is previously designated as type II enteropathy-associated T cell lymphoma (EATL). Unlike to classic form of EATL, MEITL is not associated with celiac disease. The diagnosis of MEITL is very challenging and the clinical outcome of patients with MEITL is very poor. Herein we describe a series of four patients diagnosed with MEITL identified upon a 10-year institutional retrospective review. Histopathologic examination of these cases revealed monotonous population of medium sized cells infiltrating intestinal mucosa, positive for CD3, CD8 and CD56 in all four cases. Two patients had the combination chemotherapy; however, the average survival time was only 7.5 months for these two patients after diagnosis. The aim of the present case series is to highlight the pathology, diagnosis and clinical course of the patients with MEITL based on the current literature.