Vascular diseases of the spleen are relatively uncommon in the clinical practice. However, the reported incidence has been progressively increasing, probably due to advances in the imaging modalities used to detect them. This disease condition often presents with non-specific clinical manifestations, but can be associated with significant morbidity and mortality. This review article aims to provide updated clinical information on the different vascular diseases of the splenic vasculature—splenic vein thrombosis, splenic vein aneurysm, splenic artery aneurysm, splenic arteriovenous fistula, and spontaneous splenorenal shunt—in order to aid clinicians in early diagnosis and management.