Pycnodysostosis is a rare, autosomal recessive syndrome characterized by osteosclerosis, brittle bones, stunting, and significant craniofacial changes. The objective of this study was to report a case of a 6-year-old patient with pycnodysostosis orthodontically treated and followed up until age 10 years and to discuss the risk factors, options for orthodontic treatment, and limitations involving this type of treatment, which has not yet been performed. Prevention through counseling and periodic follow-up visits is essential in eliminating factors that predispose patients to infections and fractures. New studies are necessary to establish safe and efficient orthodontic treatment plans.