Cerebellar pial arteriovenous fistula (PAVF) in adults presenting with congestive edema of the brainstem and cervical cord has rarely been reported. Here, we report such a case. A 59-year-old man presented with progressive weakness and numbness of the limbs and hiccups for 6 months. On physical examination, his limbs had grade III muscle strength, and he was unable to stand or walk. He also had occasional incontinence and retention. There was a positive Babinski sign in both lower limbs. Below the neck, he had hypoesthesia of the skin. Magnetic resonance imaging (MRI) showed edema of the brainstem and upper cervical cord. Digital subtraction angiography (DSA) confirmed there was a cerebellar nongalenic-type PAVF lesion; the feeding arteries were the branches of the posterior inferior cerebellar artery (PICA); and the draining vein was a single cortical vein that ultimately entered the venous systems of the brainstem and upper cervical cord. Casting Onyx-18 via the PICA obliterated the PAVF. Postoperatively, the patient recovered gradually. Two and a half months later, he could walk using a crutch and had no incontinence. Six-month follow-up MRI and DSA revealed recession of the edema of the brainstem and cervical cord and no recurrence of this PAVF. One year later, he was nearly normal. In conclusion and extremely rarely, cerebellar nongalenic-type PAVF can present with congestive edema of the brainstem and high cervical cord. This treatment by Onyx-18 embolization can resolve PAVF.