A retrospective study was performed to determine the incidence of spinal abnormalities in patients with skeletal defects of the upper extremities. The incidence of scoliosis in the patient population studied was 16%, and spinal abnormalities of all types were present in 18% of the population. Patients with bilateral amelia had a 100% incidence of scoliosis. Those patients with unilateral amelia had a 50% incidence of scoliosis. Patients with ulnar and radial hemimelias had an incidence of scoliosis of 20 and 13%, respectively. The transverse complete and partial hemimelics had an 18% incidence of scoliosis and the phocomelic group had an incidence of 11%. The patient population studied had an increased incidence of scoliosis compared with the general population. The patients with amelia had a particularly high incidence of scoliosis. There was no relationship between the side of the deficiency and the direction of the curve. The age of onset was not found to be related to the progression of the curve. The results of bracing were poor, due to patient rejection of the brace. All patients with upper limb deficiencies warrant close observation throughout growth for the development of scoliosis.