BACKGROUND: Isolated bilateral profunda femoris artery aneurysm (PFAA) is a very rare entity. Most of the cases are unilateral and occur with synchronous aneurysms elsewhere. Symptoms range from none to limb ischemia or hemorrhage because of rupture. METHODS: We present a rare case of PFAA. In contrast to the general rule, the patient had a bilateral PFAA which was isolated to the deep femoral artery. The aneurysm was discovered after signs of acute limb ischemia caused by distal embolization. The patient was treated surgically with open aneurysmectomy and ligation of a branch of the deep femoral artery. CONCLUSION: PFAA are asymptomatic most of the time. Surgical repair is always recommended to prevent such life-threatening complications. Different treatment modalities are offered, including endovascular options. The presence of a PFAA should prompt screening for concomitant aneurysms.