This syndrome occurs with a frequency of 1/1,00,000 in live births and can be caused by mutations of two genes, the Cockayne Syndrome Type I (CKN1) or Excision-Repair Cross-Complementing Group 8 (ERCC8) and the Excision-Repair Cross Complementing Group 6 (ERCC6), located on chromosomes 5 and 10q11, respectively [2]. The gene discussed is ERCC8; the disease is Cockayne syndrome type 1.