Osteoclastoma is a rare skeletal lesion, characterized by large multinucleated giant osteoclastic cells; this lesion usually affects young adults 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 ring-like area over the lambdoid suture, with irregular margins and calcareous deposits. The tumour was expanding mainly toward the endocranium involving both cranial tables and diploë, without infiltrating the brain parenchyma. The child underwent complete microsurgical removal of the lesion. Histopathological findings revealed the giant cell tumour osteoclastoma. Correct modern preoperative neuroimaging workup, coupled with microneurosurgical technique, allowed successful lesion removal with good outcome. A review of the more recent literature and of mechanisms of pathology together with neuroradiological protocol and results of treatment are discussed.