Esophageal Crohn’s disease (CD) is rare. We present a young girl with dysphagia, weight loss and retrosternal pain due to esophageal CD. Diagnosis was only confirmed after repeated esophageal biopsies and an additional ileocolonoscopy. Abdominal ultrasound and fecal calprotectin did support the diagnosis of CD. Treatment by exclusive enteral feeding, temporary oral viscous budesonide, esomeprazole, and azathioprin resulted in gradual remission of ulcerations. Due to loss of response after six months, maintenance therapy was switched into methatrexat and subsequently into infliximab. In literature, only a few cases have been reported. Both esophagogastro and ileocolonoscopy should be considered in case of aspecific upper gastrointestinal symptoms and despite absent lower gastrointestinal symptoms.