This article reports the diagnosis and treatment of a child with Kabuki syndrome treated for bilateral hip dislocation.A boy,aged 8 months, treated for bilateral hip dislocation, Physical examination shows bilateral hip dislocation, unusual facies, growth retardation, dermatoglyphic abnormalities. KMT2D gene mutation was found by gene detection,so,the boy was diagnosed Kabuki syndrome. By reviewing literatures, The incidence of Kabuki syndrome combined with hip dislocation in China is slightly lower than abroad. In foreign literature，most of the patients are female, bilateral hip are common, and which can be combine d with patellar dislocation.Conservative treatment can be successful reduction, but it is easy to re-dislocation.Pavlik harness treatment in neonatal period can achieve satisfactory results, simple open reduction is prone to re-dislocation, Dega osteotomy / incomplete periacetabular osteotomy combined with femoral osteotomy can achieve better results, a few patients with postoperative infection around the hip, and there is a certain probability of ischemic necrosis of the femoral head.