Eight children with mucopolysaccharidosis I (MPS I), representing 33% of all children with MPS I seen at our institution during an 18-year period, developed hypertension. Five of these hypertensive children also exhibited symptoms of aortic coarctation. The radiographic evaluation of four of these children with MPS I (three with Hurler syndrome, MPS I H, and one with Scheie disease, MPS I S) and arteriopathy affecting the thoracic aorta, abdominal aorta, and visceral and renal arteries is presented. Hypertension developed in all four children before they were 4 years old; three had differences between upper- and lower-extremity blood pressures. Irregular narrowing of the abdominal aorta with either multiple minor asymmetric wall lesions (n = 2) or abrupt concentric narrowing (n = 2) was present in all children as shown by aortography (n = 3), sonography (n = 3), MR imaging (n = 2), and/or autopsy (n = 1). A variety of other vessels also were involved, including the ascending aorta (n = 1) and vertebral (n = 1), axillary (n = 1), intercostal (n = 2), lumbar (n = 2), mesenteric (n = 3), renal (n = 2), and iliac arteries (n = 3). Autopsy in one child demonstrated thickened heart valves, narrowing of the coronary arteries, and irregularity of the aorta due to deposition of mucopolysaccharide material within the intima. Our series demonstrates various facets of the arteriopathy of MPS I as shown by sonography, MR imaging, and angiography.