Proteus syndrome is a rare congenital hamartomatous disease frequently responsible for musculoskeletal deformities. The results and complications of surgical treatment are not well documented owing to the scarcity of reported cases.

The authors report a case of poor evolution of valgus proximal tibial osteotomy in a 6-year-old girl with Proteus syndrome. The surgery was complicated by extensive deep wound necrosis exposing the tibial bone, necessitating surgical excision, antibiotherapy and controlled wound healing. At 1 year postoperatively, the deformity recurred.

The possibility of serious wound complications and of recurrence must be kept in mind when operating on a limb deformity in patients with Proteus syndrome. Potential complications should be taken into account in selecting the surgical correction technique: epiphysiodesis may be preferable to osteotomy.