Rationale: Acute mesenteric ischemia due to superior mesenteric vein (SMV) thrombosis is a rare yet potentially life-threatening emergency. Our case report explores this condition in the context of a patient with liver cirrhosis due to Wilson disease. We specifically highlight the complex derangement of the coagulative balance in liver cirrhosis., Patient Concerns: A 34-year-old female with Wilson disease-related cirrhosis presented with intractable abdominal pain, nausea, and vomiting that showed no response to antispasmodic medication., Diagnoses: A contrast-enhanced abdominal computed tomography scan and Doppler ultrasound confirmed an intraluminal filling defect in the SMV, leading to the diagnosis of SMV thrombosis., Interventions: Prompt anticoagulation, intravenous fluids, and an antibiotic were initiated. Surgical consultation recommended conservative therapy with close monitoring., Outcomes: Over the following 2 days, the patient's condition improved considerably, with almost complete resolution of her symptoms. Genetic testing identified a 4G/4G homozygous genotype of the plasminogen activator inhibitor 1 gene, associated with a higher risk of thrombosis in the vessels of internal organs. After 2 months of sustained anticoagulant therapy, a follow-up contrast-enhanced computed tomography scan revealed near-complete recanalization of the SMV, and the patient remained symptom-free., Lessons: This case underscores the importance of early detection and treatment of acute mesenteric ischemia in patients with liver cirrhosis, as well as the potential role of genetic factors in thrombosis., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)