Jaffe-Campanacci syndrome is a rare entity caracterized by disseminate
d non ossifying fibromas in association with extraskeletal congenital
anomalies: cutaneous, genital, ocular and cardiovascular. Mental retar
dation is also frequently observed. Pathological fractures are the usu
al merle of revelation. We report one case original because of causal
discovery during orthodontic check-up and because of extraskeletal ano
malies not previously described. In fact the true incidence of this sy
ndrome is probably underestimated because of unrecognized features.