A young female patient suffering from Satoyoshi syndrome had the first characteristic signs and symptoms of hair loss and progressive spontaneous intermittent painful spasms of limb muscles at age 6.5 years. Thereafter, she developed chronic diarrhea, amenorrhea, and skeletal deformities. In the orofacial region, she suffered from painful spasms of the masseter (jaw closing) muscles, progressive tooth loss, and degeneration of the mandibular condyles. Treatment with steroids and provision of complete dentures improved the signs and symptoms. Early diagnosis and timely provision of multidisciplinary care can minimize complications in these patients and improve their orofacial functions and quality of life.