Renal artery aneurysms (RAAs) are rare lesions with a prevalence of less than 1% in the general population. Renal arteriovenous malformations (AVMs) are rare lesions with an estimated incidence of less than 0.04%. The coexistence of these two clinical entities is extremely rare and narrows the available treatment options by endovascular or open surgery. We describe a case of a giant symptomatic RAA type III, which was combined with a high-flow renal AVM in the right kidney. Using two vascular plugs, the RAA was excluded successfully. The perfusion of the right kidney’s lower pole was preserved by implantation of two covered stents in the inferior segmental renal artery.

Von Gierke disease, also known as glycogen storage disease type I, co-existent with an abdominal aortic aneurysm (AAA), is an extremely rare combination of diseases that requires challenging therapeutic measures. We present, for the first time in literature, the case of a 62-year-old female with von Gierke disease who required open surgical repair of an AAA with challenging neck anatomy outside of instructions for use of endovascular repair. Even though the surgical risks for lifethreatening complications, such as pancreatitis, metabolic acidosis, and kidney failure, were high, the 6-month postoperative course was uneventful. Despite the invasiveness of the treatment, surgery to treat the AAA was safe and effective.Further data is needed to draw robust conclusions about the treatment of choice for those patients with diseases in co-existence with AAAs.