Objective: An aberrant internal carotid artery (ICA) is a rare congenital vascular malformation and many previous studies have emphasized this anomaly because of an increased risk of injury during otological surgical procedures. However, neither spontaneous massive bleeding nor infectious pseudoaneurysm associated with aberrant ICA has been reported to date. Case presentation: We present the case of a 54-year-old healthy female patient who experienced sudden massive bleeding from the ear following a single sneeze. This patient had suffered from ipsilateral otorrhea due to acute otitis media for one month. Based on a radiological examination in combination with past medical history, infectious pseudoaneurysm associated with aberrant ICA was diagnosed as the cause of bleeding. Although an embolic infarction during the endovascular procedure induced slight hemiparesis, the pseudoaneurysm was successfully managed with endovascular coil trapping. Conclusion: Although aberrant ICA has been reported as a possible cause of ear bleeding during otological procedures in the past, this is the first report of a spontaneously ruptured pseudoaneurysm associated with aberrant ICA. This case indicates that it is important to consider ruptured pseudoaneurysm in the differential diagnosis of ear bleeding, and also provides evidence that endovascular treatment is suitable for this condition.