Chorea-acanthocytosis is a rare neurological disorder that produces involuntary body movements, along with a condition of misshapen red blood cells that is characterized by appearing in early adulthood. There are numerous orofacial manifestations linked to chorea-acanthocytosis that the dental practitioner must consider in early and late stages of the disease, such as chronic oral ulcerations, chronic mouth grinding, difficulty swallowing, and biting the lip and tongue, among others. This case, the first to the authors’ knowledge to address the area of orofacial pain, provides general signs and symptoms of the disorder and management following a multidisciplinary approach. The life span of patients with this disorder is generally shortened, and correct management is essential to improve the quality of life.