The association between Arnold-Chiari type 1 malformation and hemihypertrophy has not been appreciated but a few case reports have suggested their association and proposed a common pathogenesis of dysembryoplasia of mesoderm. We report a case of 17 year-old girl presenting with left side hemihypertrophy and scoliosis. Magnetic resonance imaging (MRI) study of brain and spine revealed underlying Arnold- Chiari type 1 malformation and syringomyelia. The purpose of this paper is to emphasize the need for central nervous system evaluation in patients with hemihypertrophy. (J Korean Acad Rehab Med 2008; 32: 481-484)
