Renal osteodystrophy refers to a spectrum of bone diseases caused by pathologic alterations in the metabolism of calcium, phosphate, and bone in the context of end-stage renal disease and secondary hyperparathyroidism. Radiographic alterations affecting the jaw and facial skeleton are common and among the earliest signs of renal bone disease. Renal osteodystrophy also shares clinical, histologic, and radiologic similarities with several benign fibro-osseous conditions affecting the craniofacial region, and its recognition is critical to prevention, choice of therapy, and overall prognosis. The aim of this article is to review the craniofacial manifestations of renal osteodystrophy, describe the work-up of a patient with macrognathia and facial disfigurement caused by renal bone disease, discuss the challenges in arriving at a definitive diagnosis, and highlight an interdisciplinary approach to evaluation and timely diagnosis in overall management. Schlagwörter: end-stage renal disease, renal osteodystrophy, uremic leonthiasis ossea