Objective To investigate the short-term effectiveness of total hip arthroplasty with Wagner Cone stem combined with subtrochanteric shortening osteotomy for adult patients with Crowe Ⅳ developmental dysplasia of the hip (DDH). Methods A clinical data of 18 patients (20 hips) with Crowe Ⅳ DDH between January 2015 and June 2017 was retrospectively analyzed. There were 5 males (6 hips) and 13 females (14 hips), with an average age of 42 years (range, 20-67 years). There were 18 cases with unilateral DDH and 2 cases with bilateral DDHs. The " 4” sign and Trendelenburg sign of affected hip were positive. Preoperative Harris score and visual analogue scale (VAS) score were 41.95±6.90 and 5.05±1.15, respectively. The length discrepancy was (4.76±2.59) cm in patients with unilateral DDH. And the lengths of bilateral lower limbs in patients with bilateral DDH were equal. The acetabular anteversion angle, femoral anteversion angle, and combined anteversion angle were (32.82±2.79), (46.18±6.80), and (79.01±7.54) °, respectively. All patients were treated by total hip arthroplasty with Wagner Cone stem and subtrochanteric shortening osteotomy. The length of osteotomy ranged from 2.0 to 3.5 cm (mean, 2.38 cm). Results The operation time was 116-161 minutes (mean, 138.4 minutes); the volume of intraoperative blood loss was 600-1 200 mL (mean, 795 mL); the volume of drainage after operation was 100-630 mL (mean, 252 mL). All incisions healed by first intention. The symptom of sciatic nerve injury occurred in 1 case and relieved after symptomatic treatment. All patients were followed up 12-29 months (mean, 18.4 months). The " 4” sign and Trendelenburg sign of affected hip were negative. The Harris score and VAS score at last follow-up were 87.50±5.06 and 0.75±0.85, respectively. The acetabular anteversion angle, femoral anteversion angle, and combined anteversion angle were (16.21±4.84), (18.99±2.55), and (35.20±5.80)°, respectively. There were significant differences in above indexes between pre- and post-operation (P<0.05). The length discrepancy was (0.72±0.70) cm in patients with unilateral DDH, which was significant shorter than the preoperative value (t=7.751, P=0.000). And the lengths of bilateral lower limbs in patients with bilateral DDH were equal. X-ray films showed that the osteotomy of femur healed at 3-6 months (mean, 4.1 months) without the signs of loosening, sinking, osteolysis, and dislocation. Conclusion Total hip arthroplasty with Wagner Cone stem and subtrochanteric shortening osteotomy can obviously improve the hip joint function and restore the length of lower limb. The short-term effectiveness is satisfactory, but the long-term effectiveness and survival rate of prosthesis need to be further observed.