We report a 27-year-old Caucasian woman with Jaffe-Campanacci syndrome. She presented with multiple cafeacute;-au-lait spots and multiple non-ossifying fibromata of the bones, noted on radiographs and proven by tissue biopsies. Only the left side was involved She also suffered from moderate scoliosis, stenosis of the aortic isthmus, chylothorax. and a chylopericardium. The etiology is discussed.
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