Cavernous angiomas (cavernomas) are vascular malformations of the brain characterized by abnormal capillaries. Ventricularcavernomas are considered rare; however, an extremely unusual topography is the septum pellucidum, with only five reported casesin the English literature. These malformations may rupture and cause very large hematomas, leading to neurological impairment.Cavernomas can be familial or sporadic; additionally, these may appear following brain radiotherapy in extremely rare cases.Herein, we present an extremely rare and unique case of a septum pellucidum cavernoma that occurred in a young male who hadpreviously undergone brain radiotherapy in childhood due to acute lymphoid leukemia. Following presentation, he was diagnosedwith generalized seizures. The cavernoma was resected via an anterior interhemispheric transcallosal approach, following which thepatient remained stable without neurological sequelae.To conclude, ventricular cavernomas are rare lesions, especially when located at the septum pellucidum, thus constitute achallenging neurosurgical approach.
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