Moyamoya disease is a rare chronic cerebrovascular disorder with progressive stenosis of intracranial internal carotid arteries and their proximal branches resulting in the development of severe collateral circulations and associated with a high risk of stroke. Moyamoya disease may have extracranial manifestations such as coronary artery fistulas and stenosis, cardiac valve stenosis, congenital heart disease, and renal artery stenosis. Although organs from donors with Moyamoya disease are considered safe for transplantation, there are few reports describing the use of the organs. Here, I described the unfortunate case of kidney transplant (KT) recipient who received transplanted kidney from donor with Moyamoya disease presented with early transplant renal artery stenosis (TRAS). A 42-year-old Thai male with end-stage renal disease from unknown cause underwent KT from a 32-year-old Thai male deceased donor who was firstly diagnosed Moyamoya disease with catastrophic cerebral infarction. The KT was complicated by delayed graft function and his kidney function recovered after 2 sessions of hemodialysis. The maintenance immunosuppressive drugs included cyclosporine, mycophenolate mofetil and prednisolone. Routine Doppler ultrasound examination of transplanted kidney was done at postoperative day 7 without abnormal findings. Three months after KT, he presented with kidney allograft dysfunction, hypertension and volume overload. His serum creatinine increased from 2.08 to 5.53 mg/dL. The Cyclosporine level was within normal range. Serum BK virus and cytomegalovirus were undetectable. Kidney biopsy pathology showed diffuse ischemic tubular atrophy and interstitial edema and fibrosis without evidence of rejection. Repeat Doppler ultrasound examination of transplanted kidney revealed good transplant perfusion with a turbulent flow and a peak systolic velocity > 2 m/s at mid to distal main renal artery. Magnetic resonance angiography of transplant renal artery was also confirmed the stenosis. TRAS was diagnosed and percutaneous transluminal renal artery angioplasty (PTRA) was done without stent placement. His kidney function improved with serum creatinine of 2.53 mg/dL 2 weeks after the procedure. Then his serum creatinine increased again to the level of 5.52 mg/dL two months after the 1st procedure. PTRA was done again with renal artery stent placement. Finally, his kidney function recovered with serum creatinine of 1.72 mg/dL six month s after the 2nd procedure. Figure 2. To the best of my knowledge, this is the 1st reported case of PTRAS in KT recipient who received transplanted kidney from donor with Moyamoya disease. I believe that transplant physicians should continue using organs from donors with Moyamoya disease but must be aware of the possible extracranial complications like in my case.