Rationale:Persistent hypoglossal artery (PHA) is the second rare abnormal anastomosis of the internal carotid and vertebrobasilar arteries, and bilateral persistent hypoglossal arteries in particular have rarely been reported. This is the first case of bilateral persistent hypoglossal arteries presenting with posterior fossa transient ischemic attack (TIA).Patient concerns:We reported a 54-year old female with posterior fossa TIA due to the coexisting bilateral persistent hypoglossal arteries and left internal carotid artery stenosis.Diagnosis:The patient was diagnosed with posterior fossa TIA, bilateral persistent hypoglossal arteries and left internal carotid artery stenosis.Interventions:The patient was given aspirin 100 mg/qd and advised to avoid excessive neck movement.Outcomes:Symptoms of intermittent subjective dizziness accompanied by nausea were relieved.Lessons:Although requires no special treatment, PHA could be accompanied by hypoplasia of vertebral arteries and posterior communicating arteries and becomes the main blood supply pathway for the posterior circulation. Accurate identification and evaluation of PHA is important of ensuring the safety of carotid interventions and identifying specific types of stroke.