Résumé : INTRODUCTION Leiomyoma is the most common benign tumor of the esophagus (67-80%), it represents 0.4-1% of all esophageal tumors. PRESENTATION OF CASE An incidentally discovered gastro-esophageal submucosal tumor was found to have increased fluorine-18-fluorodeoxyglucose (FDG) uptake on positron emission computed tomography (PET/CT). After laparoscopic surgical exploration and local enucleation the tumor turned out to be a benign esophageal leiomyoma. DISCUSSION There are few reports of esophageal leiomyomas with a positive uptake on (PET/CT) and even fewer adopting our combination of a minimally invasive approach and frozen section examination as a management plan. Our approach avoided excessive morbid surgical resections and underestimation of a malignant disease. CONCLUSION We report this case hoping to expand the existing literature on the topic and to highlight the limitations of PET/CT in guiding the diagnosis and subsequently the management of esophageal submucosal tumors. © 2014 The Authors.