Reports of Parkes-Weber syndrome complicated by disseminated intravascular coagulation (DIC) are rare in the orthopaedic literature. This is a case report of a 23-year-old man who had this syndrome and who sustained a lower-leg fracture complicated by the DIC. Open reduction was not attempted because the DIC worsened after manual reduction. Amputation was rejected by the patient. Three months of continuous infusion of heparin and replacement therapy with fresh frozen plasma was done. Cast immobilization without further reductions was continued. The DIC improved and union of the fractures was observed at 2 years and 3 months after injury.