This case report details the management of a 66-year-old male with hemophilia A (HA) who presented with recurrent hematuria, and was found to have renal artery aneurysms and was subsequently diagnosed with a renal arteriovenous (AV) shunt. While the primary focus centers on the successful endovascular coil embolization of renal artery aneurysms, the concomitant presence of the AV shunt accentuates the significance of this case. Imaging techniques were crucial in the discovery of renal aneurysms and the diagnosis of the AV shunt malformation of the renal artery. This included an ultrasound, CT-angiography and digital subtraction angiography. The treatment approach employed prioritized endovascular coil embolization for its efficacy and reduced morbidity. Following the initial successful embolization, the identification of the AV shunt during subsequent embolization led to its targeted treatment. The case was also complicated by acute prostatitis that was treated medically. The patient's HA required careful administration of coagulation factor replacement therapy to control bleeding throughout the process. This case highlights the importance of reporting on the management of rare and complex pathologies to better understand and guide future treatments, especially involving this rare combination of renal AV shunts and hemophilia A.