BACKGROUND: Dural arteriovenous fistulas (DAVFs) at the craniocervical junction are rare. Clinical manifestations range from acute or chronic myelopathy to subarachnoid hemorrhage to brainstem dysfunction. We encountered 4 cases of DAVFs at the craniocervical junction with progressive brainstem dysfunction and investigated the typical magnetic resonance imaging (MRI) features using T-2-weighting imaging, susceptibility-weighted imaging, diffusion-weighted imaging, and contrast-enhanced imaging. Literature review revealed 10 case reports of DAVFs at the craniocervical junction manifesting with brainstem dysfunction. CASE DESCRIPTION: Four patients presented with DAVFs at the craniocervical junction with progressive brainstem dysfunction. Two patients underwent midline suboccipital craniotomy and C1 laminectomy, and 1 patient underwent transarterial endovascular embolization with Onyx 18 under general anesthesia. All neurologic deficits gradually improved after the operation. In the fourth case, the patient received conservative treatment and did not undergo any surgical procedure. MRI showed high signal intensity on T-2-weighted imaging, magnetic resonance angiography, and magnetic resonance venography. Abnormal dilated vessels and flow-void signs around the lesions were detected on susceptibility-weighted imaging and contrast-enhanced images. Two cases revealed no abnormalities and had improved neurological deficits than those showed on diffusion-weighted imaging. CONCLUSIONS: Susceptibility-weighted imaging, diffusion-weighted imaging, or contrast-enhanced scanning should be used during MRI examination of patients with progressive brainstem dysfunction to differentiate DAVFs at the craniocervical junction from other diseases, such as glioma or infection. Prompt diagnosis using MRI is of great significance in producing good functional outcomes of the patients.