Rationale: Hemobilia caused by arteriovenous malformation is extremely rare but could be lethal. To date, most reports have been single-case reports, and no literature reviews are available. Patient concerns: A 47-year-old man presented to the emergency department with abdominal pain and fever. He complained of abdominal pain and weight loss for the past 2 months. Diagnoses: Contrast-enhanced computed tomography and magnetic resonance imaging showed a heterogenous lesion located in pancreatic head and tumor was suspected. Interventions: Endoscopic retrograde cholangiopancreatography was performed and bleeding from papilla of Vater could be viewed. Nasobiliary drainage was placed to alleviate the pain and jaundice. Emergency laparotomy was performed due to the recurrence of severe pain and bleeding, and pancreatoduodenectomy was then performed. Macroscopic examination showed the ulceration connected with collected vessels which were located in pancreatic head and microscopic examination confirmed the presence of arteriovenous malformation. Outcomes: The patient recovered uneventfully and was discharged 10 days after the surgery. He is asymptomatic on 4-month follow up. Lessons: Arteriovenous malformation is a rare cause of hemobilia, but it could lead to life threatening bleeding. Transarterial embolization could be effective to control the bleeding temporarily, however repeated hemorrhage may occur. Surgical resection may be a better option.