Adenoid cystic carcinoma is an extremely rare case of the esophagus. We present a female patient, aged 76 years who present with dysphagia and weight loss for tire last three month. On endoscopy there was a luminal narrowing in the middle third of the esophagus. Diagnosis was challenging due to the stenosis and the tumor size. Histopathological confirmation was obtained by subcarinal fine-needle aspiration biopsy. This type of cancer is very aggressive with short survival time. Further studies are needed to define optimal treatment.