Splanchnic venous thrombosis refers to the obstructive events of the abdominal venous system (portal, splenic, mesenteric, and suprahepatic) present with or without an underlying liver disease. These are rare disorders generally associated with both local risk factors, such as intra-abdominal infections, surgery or abdominal trauma, and systemic risk factors, represented by drugs (notably estrogen therapy) and some types of inherited or acquired thrombophilia, including myeloproliferative neoplasms associated with the V617F mutation of the JAK2 gene. The clinical presentation is heterogeneous, ranging from asymptomatic patients with incidental findings on abdominopelvic imaging to severe systemic compromise in sudden onset cases, with a high morbidity and mortality burden. Anticoagulation therapy is the current recommended treatment based on observational studies, with no consensus on management in the acute and long-term setting for both inmediate complications such as acute liver failure and intestinal ischemia or those arising from secondary portal hypertension over a longer period of time.