BACKGROUND Cardiovascular diseases are very common in patients with end-stage renal disease and are the underlying cause of approximately half the deaths in dialysis patients. In those patients vascular calcifications are typically seen in the tunica media and therefore represent histopathological changes different to those in atherosclerosis. For the evaluation of cardiovascular risk in chronic dialysis patients, a method is needed to reliably identify patients who have to undergo invasive diagnostics. Coronary artery calcium assessed by electron beam computed tomography (EBT) has been suggested to measure the extent of coronary atherosclerosis in patients with normal renal function. It might also be of value in the stratification of cardiovascular risk in patients with renal failure. CASE REPORT We report the case of a 21-year-old female with end-stage renal disease as a result of idiopathic infantile hypercalcemia with nephrocalcinosis and extensive vascular calcification. Due to a total atrioventricular block, a 2-chamber pacemaker had been implanted in 1998. Because of a very high calcium score in EBT, depressed left ventricular function in echocardiography, and limited physical strength she was sent to coronary angiography. Severe 2-vessel coronary artery disease was found and successfully treated by balloon angioplasty. She was then listed for renal transplantation. Angiographic 6-month follow-up showed an excellent long-term result. CONCLUSION This case demonstrates that coronary artery disease can also be present in young dialysis patients. Discrimination of atherosclerotic vascular calcification and tunica media sclerosis typical in dialysis patients without hemodynamic effects is difficult. EBT allows for the direct detection of coronary calcium. Its extent correlates with coronary atherosclerosis and the probability of acute coronary syndromes in patients with normal renal function. This evidence has not been proved for dialysis patients, yet. However, EBT may be of value in the stratification of cardiovascular risk for patients awaiting renal transplantation.