We report the case of a 43-year-old woman who suffered from a polyostotic f
orm of Langerhans cell granulomatosis (LCG) in the mandible and maxilla. Th
e course of the disease was followed for more than 14 years. The disease's
progression finally required subtotal resection of the mandible and reconst
ruction with a microvascular fibula graft. The literature is reviewed for d
iagnostic and treatment concepts based on the different stages of LCG.