Enteropathy-associated T-cell lymphoma (EATL) has been introduced since 2001 in the World Health Organisation’s (WHO) international classification of tumours of haematopoietic and lymphoid tissues as a separate entity from T-cell lymphomas [1, 2]. The main characteristic of EATL besides its extreme rarity (less than 1% of all non-Hodgkin’s lymphomas [NHL]) [3] and its location in the intestine is that it is associated with an enteropathy and develops from the intraepithelial T-lymphocytes of the intestine. This NHL can occur as a complication of a previously recognised enteropathy or may signal its presence, and its diagnosis is thus based mainly on intestinal mucosa lesions seen at some distance from the lymphoma. The most classic form of EATL is type I (80%), which is a serious complication of celiac disease (CD). CD is the only enteropathy that is associated with this particular NHL and the molecular bonds have now been better described [4-6]. The therapeutic management of EATL remains particularly difficult and its prognosis is very poor.