Background: Ectopic cerebellar tissue located distantly from the normal cerebellum is very rare, and its pathophysiology remains to be elucidated.
Case presentation: We report an extremely rare case of intraosseous ectopic cerebellum detected incidentally at suboccipital craniotomy in a 46-year-old Japanese woman with hemifacial spasm. She had a small bone defect in the occipital bone, which contained a tiny area of soft tissue surrounded by cerebrospinal fluid connecting to the normal subarachnoid space through a dural opening. Histopathology demonstrated cerebellar cortex tissue consisting of molecular and granular cell layers.
Conclusions: This is the first report of glioneuronal ectopia within the skull bone separated from normal brain tissue, and it is important to distinguish this entity from other osteolytic lesions.
Keywords: Ectopic cerebellum; Glioneuronal ectopia; Intraosseous lesion; Occipital bone.