Introduction: Combined achondroplasia and hereditary multiple exostosis (HME) syndrome is a rare autosomal dominant inherited skeletal dysplasia. We report, for the 1sttime, a complex primary hip arthroplasty in a patient with combined achondroplasia and HME syndrome. We emphasize to the femoral and acetabular surgical concerns and difficulties of the surgical exposure and soft tissue balancing for this complex procedure.Case Report: An ambulatory 66-year-old female Caucasian with achondroplasia and HME presented with the left hip pain, progressive walk disability and limited range of hip motion due to severe hip osteoarthritis. Full cemented primary total hip arthroplasty (THA) with an impaction grafting technique was performed; posterior lip augmentation device was implanted to improve stability. At 5 years follow-up, the patient remains ambulatory and pain-free with improved range of hip motion. No signs of aseptic loosening are present.Conclusions: Cemented THA could be an efficient option to reconstruct the complex hip anatomy in patients with skeletal dysplasia.