Lhermitte-Duclós disease is an infrequent condition of which there are only 37 cases described in the literature. Although presently considered as a displasic process, its clinical presentation is of a typical posterior fossa tumour and should be treated as such. Even though it is a benign growth there are only 7 cases described with postoperative survival. The uniform pathological anatomy in all the cases presented makes the definitive diagnosis. The authors present a new case with postoperative survival and discuss its clinical presentation, treatment and origin of the disease.
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