Introduction: Abnormal localization of the right common and external iliac arteries is a rare vascular anomaly that may be associated with congenital renal or genitourinary malformations. Herein, we report a very rare case of a patient with VACTERL syndrome, having small bladder volume, end-stage renal disease (ESRD), and vascular abnormality, who also presented with an ascending aorta rupture, who was treated with ileobladder technique and renal transplantation (RT).
Case Presentation: A 15-year old male patient applied to our hospital with bladder volume problems in addition to VACTERL syndrome and was found to have abnormal right common and external iliac arteries during pre-operative imaging. Vascular supply to the right lower limb was derived from an anomalous branch from the left internal iliac artery which took on a convoluted course across the pelvis.
The patient was on dialysis. First, we applied the ileobladder technique, after which time the patient presented with ascending aorta rupture that we treated with a graft. 6 weeks later, we performed a living-related right kidney transplant  from the father, which was implanted on the left side of the iliac fossa by using an abnormal right iliac artery and normal left external iliac vein. The ureter was then implanted directly to the bladder. The patient is doing well.
Conclusion: RT can be successfully performed on patients with VACTERL syndrome in spite of small bladder volume and major vascular problems, such as rupture of aorta and abnormal localization of the iliac artery.