Drug-induced enterocolitis syndrome (DIES) is the term used to describe reactions occurring after drug exposure. It is a non-IgEmediated hypersensitivity reaction, characterized by symptoms that typically resemble those of FPIES (food protein-induced enterocolitis syndrome). It is a rare and probably underestimated entity, since very few cases have been reported in literature so far. To date, seven cases of DIES have been described, four in children, [1][2][3][4] one in an 18-year-old male patient, 5 and two in adult patients. 6,7 In 2014, Novembre et al 1 described the case of a 6-year-old girl who developed gastrointestinal symptoms of vomiting, diarrhea, pallor, and lethargy 2 hours after a drug provocation test (DPT) with amoxicillin (AMX). As the clinical and laboratory features, that is, vomiting, neutrophilia, methemoglobinemia (MethHb), and need for intravenous fluid support, resembled those of FPIES, the term DIES was proposed for the first time. In the following, five other cases of DIES have been reported, summarized in Table 1.