Eosinophilic enterocolitis is an exceptionally rare disease with few cases described in the literature. It is the least frequent manifestation of the broad spectrum of gastrointestinal eosinophilic diseases. We describe a case of a female patient presenting with a form of the disease manifested by Koenig's syndrome and diarrhea. This posed a diagnostic challenge for us due to its non-specific symptoms, associated with the absence of standardized histological criteria, hence the need for a high level of suspicion. There is also no consensus regarding the treatment. It should be individualized according to the age of the patient and the severity of symptoms.