Two cases of atlantoaxial subluxation secondary to occipitoatlantoaxial malformation in the domestic ferret are described. Clinical signs included cervical pain, tetraparesis and tetraplegia. Diagnosis was made using cervical radiographs, CT and, in one case, MRI. Nonsurgical management was initiated including application of a cervical brace. Motor function improved initially in one ferret but both ferrets were later euthanased due to deterioration or lack of further improvement in clinical signs.