Congenital dislocation of the knee is very rare skeletal deformity and was firstly described by Chatelaine in 1822. Since then there have been a few number of case reports and some series discussing the etiology and treatment. The tibia is displaced anteriorly in relation to the femur. It is generally subdassified as simple hyperextention, subluxation, and dislocation depending on the degree of the joint displacement and the severity of disease. Early gentle manipulation and serial splintage or plaster cast are recommended for treatment and were successful in the majority except the case of quadriceps contrarture or late correction. The authors experienced one case of congenital dislocation of the right knee and brief review of the related literatures was made.