Isolated internal iliac artery aneurysms (IIIAAs) are extremely rare. We present the case of a 75-year-old white Caucasian male patient admitted for right lower limb deep venous thrombosis and low-risk pulmonary embolism due to the compression of the external iliac vein from an IIIAA. A review of the literature was also performed to identify the epidemiologic, clinical characteristics and diagnostic and operative strategies of this disease. At present, a recommendation for the best operative strategy in the presence of an IIIAA is difficult. Our analysis demonstrated controversial results, but we are confident to recommend the endovascular treatment in an elective setting.