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Successful preoperative diagnosis and minimally invasive surgery of bile duct schwannoma. Bile duct schwannomas are rare benign tumors that frequently mimic more life-threatening tumors. Till date, only a few of these tumors have been diagnosed preoperatively. Herein, we report the case of a 68-year-old man who presented with a 1-day history of epigastralgia. The patient was preoperatively diagnosed with bile duct schwannoma using transpapillary biliary forceps biopsy and underwent local resection without major complications. The findings of our study indicate that endoscopic tumor biopsy may be helpful in diagnosing bile duct schwannoma before surgery; thus, enabling surgeons to plan an appropriate surgery while avoiding overtreatment.