A 10-year-old female spayed Dalmatian was evaluated for progressive cervical scoliosis and stiffness. This imaging report describes the imaging and postmortem findings for this patient. A diagnosis of an atypical manifestation of diffuse idiopathic skeletal hyperostosis (DISH) was made based on imaging and additional diagnostics. This report serves to increase awareness of DISH in the veterinary community, as well as to describe a unique presentation of the disease with atypical lesion distribution and severity. In addition, this report contrasts the pathophysiology and imaging characteristics of DISH with spondylosis deformans, which can appear comparable radiographically.