Melorheostosis is a benign, rare, congenital disorder of hyperostosis of one or more bones. In this report, we describe a 5-year-old girl with melorheostosis of the left iliac wing, femur, and tibia who developed severe hypertension secondary to left renal artery stenosis. Numerous soft tissue and vascular anomalies have been reported in patients with melorheostosis. To our knowledge this is the first case where renal artery stenosis has been associated with melorheostosis. Several hypotheses for bone and vascular involvement in melorheostosis are reviewed.