Spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA) is a rare hereditary disorder with a prevalence of 1/1,000,000, which affects men and women equally. It usually starts in early childhood with walking gait instability, short stature, joint pain, and limited mobility, with the symptoms progressively worsen. Clinical examination can reveal osteoporosis, scoliosis, hip degeneration, and even femoral head necrosis. This article reports an adult with SEDT-PA. The patient limped with bone pain. After admission, relevant examinations were completed, and the multidisciplinary team discussion was organized. Combined with clinical manifestations, imaging tests, and genetic tests, the final diagnosis was SEDT-PA. Bilateral total hip arthroplasty was then performed, and the lameness improved after surgery.