Background: Sylvian fissure arteriovenous malformations are rare but important vascular lesions, whose importance lies in both haemorrhage and seizure risk. Although surgery has been recommended as a treatment, the overall estimation of success has not been reported to render outcomes easier to understand in comparison to other treatment modalities. Objectives: This systematic review of the literature and two cases aims to illustrate the results of surgery as a contemporary treatment option and present a novel anatomical classification system for Sylvian fissure arteriovenous malformations. Materials and methods: A systematic review was performed by searching MEDLINE (PubMed), EMBASE and Cochrane electronic bibliographic databases from conception to 2018. The following keywords were used: ‘Sylvian fissure’ AND ‘AVM’ OR ‘arteriovenous malformation’ OR ‘intracranial arteriovenous malformation’ OR ‘cerebral arteriovenous malformation’ OR ‘brain arteriovenous malformation’. The search strategy was not limited by study design but only included keywords in the English language. In addition, two local institution Sylvian fissure arteriovenous malformations are presented and incorporated. Results: A total of nine full-text articles were included in the analysis. The results of reported cases and the literature review emphasise the role of surgery in the treatment of Sylvian fissure arteriovenous malformations, with an acceptable result in carefully selected patients. We propose a classification system which may inform the choice of surgical approach for these lesions. Conclusions: Surgery remains the cornerstone of Sylvian fissure arteriovenous malformation treatment, which may apply to high-grade lesions in this special anatomical location.