There is little information concerning long-term outcome of vertebral agenesis. In this series of eight patients with variable severity of vertebral agenesis, three died during infancy, two of associated congenital anomalies involving the cardiorespiratory system and one of unknown causes. The remaining five have had no life-threatening problems. They have normal intellect, and the developmental achievements are adequate for their age. The five patients with the highest lesions were those born to diabetic mothers. We also discuss current concepts of comprehensive team management of such infants.