A 12-year-old boy is described with bilateral radial club hands, scoliosis, hypospadias, isolated dextrocardia, hypoplastic ribs, an ectopic kidney and spina bifida occulta. Although some of the clinical features of this patient are seen in VATER association and sacrococcygeal dysgenesis, the presence of dextrocardia, facial dysmorphism, radial, renal and vertebral anomalies preclude these diagnoses.