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The incidence of hip dislocation in Kabuki syndrome (KS) is thought to be between 18% and 40%. Although hip dislocation is apparently well recognized in KS, to our knowledge, its management and outcome have not previously been addressed in the literature. We report 2 children (3 hips) with KS and hip dislocation who underwent surgical treatment with a minimum of 24-month follow-up. In addition, we review the current literature on the treatment of orthopaedic problems in KS.