We report the case of a patient with long-term history of hypertension, presenting with transient neurological disorders and severe graft failure several years after kidney transplantation. Cause of end-stage renal disease was hypertensive nephrosclerosis. Chronic hemodialysis lasted for 1 year. After transplantation and throughout follow-up, serum creatinine ranged from 200 to 230 μmol/L and maintenance immunosuppression included sirolimus and low-dose steroids. Six years after transplantation, the patient presented with right hip pain radiating to the lower back, transient aphasia, confusion, and hemiparesis. Surprisingly, progressive anuria was established requiring dialysis. After numerous nonconclusive investigations including renal histology, a contrast computed tomography scan discovered a Stanford B aortic dissection from the left common carotid artery and left subclavian artery to bilateral internal and external iliac arteries, including the right femoral artery. No surgical treatment was opted and hemodialysis, tight control of blood pressure and oral anticoagulation were established. Immunosuppression was lightened to low-dose steroids alone. After 8 months, chronic dialysis was stopped, and today, 22 months after the diagnosis of aortic dissection, the patient is doing well with a still functioning graft (creatinine, 377 μmol/L; modification of diet in renal disease-glomerular filtration rate, 15 mL/min), and without any other immunosuppression than low-dose steroids.