The orthodontic diagnosis and comprehensive management of a 12.5-year-old Saudi girl, diagnosed with Schwartz-Jampel Syndrome (SJS) is presented. SJS is a rare autosomal recessive disorder that affects musculoskeletal structures of the body with clinical manifestations in the maxillofacial region. While few reports in the literature have discussed the dental aspects of the syndrome, this paper attempts to describe the full course of a two-phase comprehensive orthodontic treatment, and discuss its findings.