Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder affecting the connective tissue, resulting in laxity, and can be challenging if surgical treatment is needed. Literature concerning scoliosis and its treatment in LDS is limited. This is a report of scoliosis surgery in a 12-year-old girl with LDS. She underwent anterior instrumented spinal fusion of the segments T12 to L3 because of a left-sided thoracolumbar scoliosis of 42° with dysplastic pedicles. The scoliosis was reduced by 40%, from 42° to 25°, resulting in a satisfactory clinical outcome with a minimal amount of surgical invasion and lack of complications. A minimal approach and anterior instrumented spinal fusion surgery can be considered a valuable surgical treatment alternative for scoliosis in patients with LDS, avoiding the placement of pedicle screws in dysplastic pedicles and using the abnormal laxity of the connective tissue as an advantage.