Congenital dislocation of the knee (CDK) is a rare anomaly. In CDK, there is variable anterior displacement of the tibia under the femur. It commonly is associated with developmental dysplasia of the hip, foot deformity, and syndromes such as arthrogryposis and Larsen syndrome. Many etiological factors have been considered without consensus. The pathognomonic feature of congenital dislocation of the knee is fibrosis and shortening of the quadriceps muscle with hypoplasia of the suprapatellar pouch. Management of congenital dislocation of the knee should be started soon after birth by gentle manipulation and serial casting in progressive flexion until right-angle knee flexion is achieved. At this point radiographic reduction of the tibia under the femur needs to be documented. In cases of failure of conservative treatment or late presentation, mini-open quadriceps tenotomy or open V-Y quadricepsplasty may be necessary. When multiple deformities are present in same limb, priority is given to reduction of the knee joint before managing hip or foot deformity. On longer follow-up, these children function well but may have a persistent extensor lag, flexion deformity, and occasionally progressive genu valgum. Need for long-term follow-up and secondary intervention should be considered vital during parental counseling.