The authors observed the spontaneous rupture of a dysplastic superficial femoral artery in a 47 years old patient with Von Recklinghausen disease. The patient presents a gigantism of his right lower limb. On angiography, the femoropopliteal axis is polyaneurysmal and there is evidence of rupture at mid-thigh. The diseased artery is excluded by interposition of a dacron prosthesis, with reimplantation of the deep femoral artery. Histology shows fibrohyalin thickening of the intima. Dysplasia of large limb arteries is excessively rare in Von Recklinghausen disease.