Introduction: Inflammatory myofibroblastic tumour of the common bile duct (CBD) is an extremely rare low-grade malignancy with various biological behaviours and a lack of specific clinical and histopathological features. Preoperative and intraoperative diagnosis are challenging, and an undiagnosed tumour can cause increased surgical trauma. Case presentation: We report a 34-year-old male who presented with yellowish whole-body skin and yellowish eyes for 20 + days. There was no history of abdominal swelling or pain, fever or chills. Computed tomography (CT) revealed a well-defined, asymmetrical, circumferential obstructing mass in the hilum-mid CBD. The patient was suspected to have cholangiocarcinoma and underwent total bile duct tumour resection and digestive tract reconstruction. The postoperative pathological diagnosis was inflammatory myofibroblastic tumour, and the patient was followed up for three years and exhibited good health. Conclusion: We report a case and literature review regarding inflammatory myofibroblastic tumour of the CBD, which is an extremely rare low-grade malignancy.