Bone involvement is relatively rare in vascular malformations. Gorham-Stout disease, also referred to as vanishing bone disease, is characterized by osteoclast activation and osteolysis caused by proliferating lymphatic endothelial cells. We present the case of a 12-year-old boy who had Gorham-Stout disease at the age of 8 years. The clinical course was complicated by pathological fractures and localized intravascular consumption coagulopathy. Sclerotherapy and embolization therapy led to normalization of the coagulation parameters and significant improvement of the clinical findings. We speculate that this effect may be attributable to the elimination of lymphatic endothelial cells.