A 17-month old female spayed Cavalier King Charles Spaniel presented for suspected caudal occipital malformation syndrome. She had, over the past few months, started showing neurological signs consistent with such a diagnosis and because of the high prevalence in the breed, the owners requested a thorough work-up. On presentation, the dog had exhibited pain along her cervical spine, a left head tilt, and started scratching at the back of her head incessantly. She was initially managed with pain medication by the referring veterinarian but her clinical signs waxed and waned prompting her arrival to Cornell University's Hospital for Animals Neurology Service. Radiography, MRI, and CT scans were all consistent with syringomyelia secondary to caudal occipital malformation syndrome. Because of the better long term prognosis with surgical intervention1 the patient underwent foramen magnum decompression with cranioplasty and was managed postoperatively with appropriate analgesia (gabapentin). Although the patient experienced some anticipated post-operative complications, each subsequent follow-up examination revealed improvement to her clinical signs.