Osteoclastoma is a rare skeletal lesion, characterized by large multin
ucleated giant osteoclastic cells; this lesion usually affects young a
dults with a prevalence of 1 case/1 million population. We report the
case of a 9-year-old girl with a right temporal tumescence: X-ray, CT
and MRI revealed the presence of a right temporal hyperostotic ringlik
e area over the lambdoid suture, with irregular margins and calcareous
deposits. The tumour was expanding mainly toward the endocranium invo
lving both cranial tables and diploe, without infiltrating the brain p
arenchyma. The child underwent complete microsurgical removal of the l
esion. Histopathological findings revealed the giant cell tumour osteo
clastoma. Correct modern preoperative neuroimaging workup, coupled wit
h microneurosurgical technique, allowed successful lesion removal with
good outcome. A review of the more recent literature and of mechanism
s of pathology together with neuroradiological protocol and results of
treatment are discussed.