Cockayne syndrome is a progeria syndrome characterized by skin photosensitivity, growth retardation, visual and auditory loss, degeneration of the nervous system and accelerated aging. In this paper, we introduced the aging manifestations of the syndrome in multi-system organs, reviewed its pathogenic mechanisms of defects in transcription and transcription-coupled nucleotide excision repair, and discussed possible key roles of base excision repair and mitochondrial dysfunction in the pathogenesis. We further elucidated the research progress of targeted therapy for Cockayne syndrome in combination with the researches concerning the pathogenic mechanism of aging.