A 23‐month‐old castrated male Cavalier King Charles spaniel was evaluated because of a 6‐month history of unusual rippling/undulating movements of the right facial muscles that were continuous and persisted during sleep.Neurological examination revealed narrowing of the right palpebral fissure and unilateral right‐sided facial myokymia that was characterised by myokymic, and to a lesser degree, neuromyotonic discharges on concentric needle electromyographic examination.After persisting unchanged for almost 2.5 years from its onset, the facial myokymia gradually disappeared over a 6‐month period concomitant with the emergence of a persistent ipsilateral facial paralysis and head tilt. At 5 years and 9 months after the first examination, signs of ipsilateral lacrimal, pharyngeal and laryngeal dysfunction became evident and the dog was euthanased. Postmortem examination identified a malignant (WHO grade III) meningioma in the right cerebellopontomedullary angle that compressed the ventrolateral cranial medulla, effaced the jugular foramen and internal acoustic meatus and extended into the facial canal of the petrous temporal bone.Novel findings were the unique observation of isolated unilateral facial myokymia preceding diagnosis of a meningioma affecting facial nerve function within the caudal cranial fossa and the remarkably long duration of neurological signs (75 months) attributable to the neoplasm.