Klinefelter syndrome is a chromosomal condition in which there is a presence of extra X chromosome in the males. It is characterized by small testicles, azospermia, gynecomastia, several general, craniofacial, oral manifestations and radiological manifestations. This review highlights the etiology, salient clinical features, features during the life span, other manifestations reported, oral and craniofacial radiological features, investigations, treatment, recent advancements in the management, risk factors of Klinefelter syndrome are discussed briefly.