The association between kidney and liver polycystosis and arterial aneurysms is well documented. However, it remains unclear whether these patients are at increased risk of malignant transformation.
In this article, we describe a case of a primary angiosarcoma of the femoral artery with metastatic spread into the lungs and hilar lymph node arising in a 74-year-old man with kidney and liver polycystosis and multiple arterial aneurysms.