We report the case of an 11-year old girl who presented with an intracranial chondromyxoid fibroma arising from parietal bone. Chondromyxoid fibroma is a benign cartilaginous neoplasm, which can be easily mistaken for chondrosarcoma, especially in small biopsies. It is more common in long bones and is least common in cranial bones. Published work describes about 33 cases occurring in cranial bones with almost equal incidence in the bones of the base of the skull and cranial vault. To our knowledge, this is the second case of chondromyxoid fibroma reported to occur in parietal bone and is the first case reported in an English-language journal. We present this case for its rare anatomical, clinical, radiological and histological presentations.

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