Background: Brachiocephalic vein tumor is a rare vascular malformation, which is often reported in case reports. At present it has attracted much attention due to the serious complications, such as vein tumor rupture, pulmonary embolism, venous thrombosis, etc. We report a case of left brachiocephalic vein tumor with compression symptoms.Case presentation: a 52-year-old male who was admitted to our hospital with irritating cough for more than one month. Chest contrast-enhanced CT showed a localized expansion of 5.2cm in diameter of the left brachiocephalic vein in the anterior mediastinum. The patient received venous angioplasty with brachiocephalic vein tumor resection, and the postoperative recovery was well.Conclusion: Surgical operation is an effecive treatment method for brachiocephalic vein tumor, but it is still necessary to choose the appropriate way according to the type, size, location, lesion scope and complications of the vein tumor.