Background Giant aneurysms of the intracranial vertebral artery are very rare cerebrovascular lesions. Due to the rarity of these aneurysms, we know little about them. Methods We performed a systematic review of the English literature by searching the PubMed database. The inclusion criteria were as follows: (a) the full text was available and (b) complete clinical data were available. Results A total of 45 articles were identified, containing 53 patients (53 aneurysms). The patients were aged from 5 to 77 years (48.8 ± 20.8 years). Four patients receiving conservative treatment died. The remaining 49 patients were divided into the aneurysm removal group ( n = 17) and the aneurysm reserve group ( n = 32). The outcomes of the 49 treated cases could be obtained in 45 cases, 31 of which (68.9%, 31/45) had a Glasgow outcome scale score of 4–5. Conclusions It is still difficult to treat intracranial giant vertebral artery aneurysms, regardless of the treatment selected. Because of the malignant natural history, aggressive treatment is still advocated.