Adenoid cystic carcinoma of the esophagus is an exceptionally rare malignancy with a submucosal growth pattern, which makes diagnosis by conventional endoscopy difficult. The case concerns a 47-year-old man in whom a routine examination revealed an esophageal submucosal lesion. Endoscopic ultrasound (EUS) precisely localized it in the submucosal layer as a hypoechoic mass with a sieve-like appearance and an intact muscularis propria. The tumor was completely removed by endoscopic submucosal excavation (ESE) and histopathology confirmed adenoid cystic carcinoma of the esophagus. During 5 years of regular follow-up, the patient was in excellent condition without recurrence or metastases. This case highlights the role of EUS in early stage diagnosis and staging and endoscopic resection as a minimally invasive alternative to esophagectomy in selected patients.