Giant splenic artery aneurysms are extremely rare entities that have important clinical implications. The size and the natural history pose unique challenges in the management of these lesions. We present one such case that was associated with a primary arteriovenous malformation in the splenic hilum. This is the third largest aneurysm reported in literature so far and the characteristic feature is that this is the first case of a hilar arteriovenous fistula complicated by formation of a giant aneurysm and another smaller aneurysm. In our opinion the hilar malformation was congenital in origin and responsible for the formation of the two aneurysms. We also present an up to date review of literature on this subject.