We report the case of a 43-year-old woman with a medical history of active smoking who presented with abdominal pain that had appeared 4 months ago. The physical examination revealed periumbilical pain associated with weight loss. Laboratory studies revealed elevated inflammatory markers (C-reactive protein: 54 mg/dL) and hypoalbuminemia (22 g/L). Abdominal computed tomography (CT) and magnetic resonance enterography (MRE) showed a thickening of the small bowel at the junction between the jejunum and ileum, at 30 cm, suggesting Crohn’s disease. Gastroscopy showed an appearance of duodenal villous atrophy, confirmed by Marsh stage 3c biopsy findings. Anti-transglutaminase antibodies were positive at 17 U/ml. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) detected a jejunoileal accumulation of 18F-FDG, with a standard uptake value (SUV) at of 8.1. Small-bowel capsule endoscopy confirmed the appearance of villous atrophy with the presence of sporadic ulceration ([Fig. 1]), then a completely ulcerated jejunoileal segment over several tens of centimeters, with necrotic pseudomembranous and hemorrhagic spots ([Fig. 2], [Video 1]). Biopsies of this ulcerated area were performed with double-balloon enteroscopy ([Fig. 3]), histological examination revealing refractory type 2 celiac disease (RCD) characterized by an increased clonal population of abnormal intraepithelial lymphocytes (IEL). Abnormal IEL phenotype was described with disappearance of the classical markers such as CD4 or CD5 and the presence of NKp46 marker, but without any sign of high-grade lymphoma ([Fig. 4]). Systemic corticosteroid therapy was initiated but did not improve the patient’s symptoms. She developed an occlusive syndrome requiring surgical resection of the ulcerated jejunoileal segment (39 cm). The surgical follow-up was simple. The diagnosis of type 2 RCD without high-grade lymphoma was confirmed. Gluten-free diet and open-capsule budesonide treatment were initiated with close surveillance of the patient due to the high risk of progression to T-cell lymphoma.