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We review the case of a 44-year-old man with Camurati-Engelmann disease, who presented with chronic right hip pain that did not improve following intra-articular hip injections. He was functionally debilitated because of the worsening pain. Routine radiographs demonstrated severe right hip osteoarthritis and severe diaphyseal sclerosis of the femur. To address the narrowed medullary cavity, appropriate reaming of the diaphysis and broaching to fill the metaphysis were performed. The patient underwent an uncemented total hip arthroplasty that resulted in an excellent recovery with no complications.Uncemented total hip arthroplasty serves as a good option for patients with hip osteoarthritis secondary to Camurati-Engelmann disease. Anticipation of potential operative challenges is the key to avoiding complications and achieving an optimal, durable outcome.