A 10-month-old female spayed Australian shepherd was presented to Cornell University Hospital for Animals' Dentistry and Oral Surgery Service for evaluation of suspected enamel hypoplasia. The patient had been presumptively diagnosed with distemper earlier that year by a neurologist based on the appearance of classic neurologic signs (including paresis, myoclonus, and involuntary chewing movements of the jaw) in conjunction with enamel defects and a history of respiratory illness. General physical examination revealed localized involuntary twitching of the muscles of the face and involuntary chewing movements of the jaw, but was otherwise unremarkable. Diagnostic workup included anesthetized oral examination, periodontal probing and charting, and full-mouth intraoral radiographs. Full-mouth dental extractions were performed without complication. This paper will use a case-based approach to discuss relevant tooth anatomy, the etiology and types of enamel hypoplasia, its corresponding differential diagnoses, clinical and radiographic features of disease, potential treatment options and important considerations. It will also briefly discuss root hypoplasia and unique features of the case.