Acute superior mesenteric venous thrombosis is rare. With advance in CT venography, angiography and diagnostic laparoscopy, the incidence of acute superior mesenteric venous thrombosis has increased worldwide with more access to early diagnosis. The use of anticoagulation medication, interventional radiology, and damage control approach has resulted in better clinical outcomes. At present, the new step-up approach for acute superior mesenteric venous thrombosis includes CT venography as the main diagnostic technique, anticoagulation as the cornerstone of therapy, local transcatheter thrombolytic therapy as the key recanalization method, and adjunctive use of arterial spasmolysis and various endovascular manipulation and damage control surgery by intestinal resection plus jejunostomy and ileostomy or open abdomen. This strategy may further improve clinical outcomes. This review will present the most recent advance in this strategy.