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Background Doege–Potter syndrome, characterized by solitary fibrous tumors and non‐islet cell tumor hypoglycemia, is rare. Here, we report a case of Doege–Potter syndrome in which retroperitoneal tumor resection was performed with continuous intraoperative blood glucose monitoring. Case presentation A 37‐year‐old man presented with hypoglycemia‐related symptoms, and a 10 × 12 × 9 cm tumor was found in his right kidney. Following tumor resection, insulin secretory abnormalities improved, and intraoperative blood glucose monitoring showed no hypoglycemic events. High levels of insulin‐like growth factor‐II confirmed the diagnosis of an insulin‐like growth factor‐II‐producing tumor with non‐islet cell tumor hypoglycemia. Postoperative serum insulin‐like growth factor‐II levels normalized, with no recurrence observed over 3 years. Conclusions This case highlights the rarity of primary retroperitoneal Doege–Potter syndrome, emphasizes the safety of intraoperative blood glucose levels during surgery, and suggests rapid recovery of insulin secretion postoperatively.