Enteropathy type T-cell lymphomas (ETL) are the most frequent T-cell lymphomas arising in the gastrointestinal tract. Commonly, the neoplasm is clinically associated with symptoms of malabsorption, and it frequently manifests as a spontaneous bowel perforation. Among ETL, two types can be distinguished by morphology, immunophenotype and, possibly, by pathogenesis. A total of 80% of ETL are characterized by a close association with celiac disease, pleomorphic cytomorphology and the rare expression of CD8 and CD56. In contrast, 20% of ETL show a monomorphic small to medium size cytomorphology and frequent expression of CD8 and CD56, an association with celiac disease is rare in the latter cases. Genetically, ETL is characterized by frequent and recurrent chromosomal gains of 9q33-q34.