The authors report a case of true isolated atherosclerotic aneurysm of the profunda femoris artery. On the basis of a careful search of the literature some aspects of this rare disease are illuminated in terms of its low incidence, pathologic background and treatment; the last should always be aggressive due to the high possibility (about 50%) of major complications mainly represented by rupture. Simple aneurysmectomy without flow re-establishment may be allowed only if the femoropopliteal tract is normal