Carotid-jugular fistula is a rare presentation of arteriovenous fistula. A case of a 60-year-old Chinese man who presented with iatrogenic carotid-jugular fistula with multiple fistulous points was reported. His presenting complaint was a gradually enlarging right pulsatile neck mass complicated by worsening symptoms of congestive cardiac failure. He had recent mitral valve annuloplasty, and a right internal jugular central venous pressure monitor insertion was performed then. Angiography revealed right carotid-jugular fistula with feeders from the external carotid, internal carotid and right vertebral arteries, all draining into the right internal jugular vein. He underwent embolisation twice resulting in transient improvement in clinical symptoms, and surgical resection was later performed in view of residual arteriovenous shunting and gradual clinical deterioration. Following surgery, he was discharged and resumed work as a janitor with no recurrent symptoms for 3 years now. In this report to be added into the literature, we discuss a rare case of iatrogenic carotid-jugular fistula with multiple fistulous points which required embolisation and subsequently surgical resection.