Background: Cavernomas are rare central nervous system (CNS) lesions that constitute a distinct type of vascular malformation encountered in the brain parenchyma or ventricular system. A cavernoma can be familial or sporadic forms and exhibit a range of presentation from incidental findings to seizures, headaches, hemorrhage. Septum pellucidum cavernoma is exceedingly rare and should be studied for its unique topographical location and clinical course. Method: We performed a comprehensive literature search and review using multiple databases. the title or abstract and MeSH keywords used included 'cavernoma,' 'cavernous hemangioma,' 'cavernous malformation,' 'cavernous angioma,' 'CM,' 'septum pellucidum,' 'SP' and 'intraventricular,' along with 'AND' and 'OR' operators. Demographic and clinical data of each patient were collected for qualitative synthesis. Result: Reported cases were diagnosed at a median age of 42 years; the most frequent symptom was headaches. The incidence of hemorrhage and hydrocephalus was 30%. Gross total resection was performed in 100% of patients and exhibit clinical improvement. Conclusion: The unique location of the cavernoma exhibits clinical presentations seen and the surgical approach used. Gross total resection conveys the impression of optimum management strategy and leads to a magnificent outcome in most cases.

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