Objective To explore the effectiveness of staged treatment of open Pilon fracture combined with soft tissue defect. Methods Between June 2007 and December 2012, 18 cases of open Pilon fracture combined with soft tissue defect were treated. There were 14 males and 4 females with an average age of 35 years (range, 19-55 years). The causes of injury included falling from height in 12 cases, traffic accident in 4 cases, and crushing by machine in 2 cases. According to AO classification, 1 case was classified as type B2 fracture, 3 cases as type B3 fracture, 5 cases as type C1 fracture, 5 cases as type C2 fracture, and 4 cases as type C3 fracture. Sixteen cases accompanied by fibular fracture (14 cases of simple fibular fracture and 2 cases of communicated fibular fracture). According to Gustilo classification, the soft tissue injuries were all type IIIB. In first stage, debridement and vaccum sealing drainage combined with external fixation were performed; open reduction and internal fixation of simple fibular fracture were used. In second stage, open reduction and internal fixation of Pilon fracture and communicated fibular fracture were performed, and the flaps of 6 cm × 5 cm to 18 cm × 14 cm were applied to repair soft tissue defect at the same time. The donor site was repaired by skin graft. Results Partial necrosis occurred in 2 flaps, the other 16 flaps survived completely. The incisions of donor sites healed by first intention, the skin graft survived completely. The average follow-up interval was 12 months (range, 6-24 months). The X-ray films showed that the bone healing time ranged from 5 to 8 months (mean, 6 months). No internal fixation failure was found. At last follow-up, the average range of motion of the ankle joint was 37° (range, 26-57°). According to the American Orthopedic Foot and Ankle Society (AOFAS) scale, the average score was 80.2 (range, 72-86). Traumatic arthritis occurred in 2 cases (11%). Conclusion The staged treatment has the advantages of accurate evaluation of soft tissue injury, shortened cure time, good reduction of the articular surface, and reduced incidence of infection, so it is an optimal method to treat open Pilon fracture combined with soft tissue defect.