Congenital dislocation of the patella is a rare condition characterized by lateral dislocation of the patella that is irreducible without surgical correction. Although there is no clear inheritance pattern, it is associated with several congenital syndromes. Patients often demonstrate flexion contracture, loss of active knee extension, increased tibial external rotation, and absent patella in the trochlea. Treatment requires surgical management and is comprised of lateral release, medial stabilization, quadriceps lengthening, and distal realignment. Results are generally favorable after treatment; persistent flexion contracture and redislocation are the most common complications. Further study is needed to define the optimal timing and treatment strategy for this uncommon condition.