Pancreatogenic hypoglycemia is a condition of relatively low prevalence but, once detected, it should be treated promptly to prevent irreversible brain damage, and surgery remains the first option in treatment of this condition. At present, the modalities in preoperative diagnosis of this condition, especially in localization techniques is continually evolving, and the treatment methods, except for surgical resection, include anhydrous alcohol injection, radiofrequency ablation, interventional embolization, and most recently, high-intensity focused ultrasound ablation therapy also achieved favorable results in treatment of pancreatogenic hypoglycemia. In this paper, the authors present the progress on surgical diagnosis and treatment of this condition, and propose the direction of future research.