More than 250 cases of Cornelia de Lange syndrome have been reported in the medical literature, but not have described the use of hip reconstruction to correct the congenital dysplasia that may be associated with this condition. This article reports the application of a bipolar hemiarthroplasty and acetabular allograft reconstruction for a 32-year old woman with congenital dysplasia and degenerative joint disease of the hip secondary to de Lange's syndrome. On admission, she was in considerable pain and unable to bear weight on the affected extremity. Her Harris hip score was 25. Following surgical intervention and a well-designed plan of rehabilitation, the patient's functional status improved markedly. Her Harris hip score was 72, and her pain was alleviated.
