Background: Portal vein thrombosis (PVT) has been reported infrequently in dogs. Objectives: To characterize the presentation, associated disease conditions, and outcome in dogs with PVT. Animals: Client-owned dogs with a diagnosis of PVT and a complete medical record. Methods: Records were retrospectively analyzed for presentation, history, physical examination, clinicopathologic data, diagnostic imaging, treatment, and outcome.Results: Thirty-three dogs were included. Common clinical signs were vomiting, diarrhea, abdominal pain, ascites, and signs of hypovolemic shock. Associated disease conditions included hepatic (14/33), neoplastic (7/33), immune (5/33), and infectious (4/33) diseases, protein-losing nephropathy (3/33), hyperadrenocorticism (2/33), protein-losing enteropathy (1/33), and pancreatitis (1/33). Fourteen dogs were receiving glucocorticoids at the time of diagnosis. Twenty-nine dogs had at least 1 predisposing condition for venous thrombosis, and 11 had 2 or more. Thrombocytopenia (24/33), increased liver enzyme activity (23/33), and hypoalbuminemia (20/33) were common laboratory abnormalities. Clinical syndromes at the time of PVT diagnosis included shock (16/33), systemic inflammatory response syndrome (SIRS), (13/33) and disseminated intravascular coagulation (3/33). Twenty-four dogs had acute and 9 had chronic PVT. Multiple thrombi were found in 17/33 dogs. Nineteen dogs survived to discharge. Dogs treated with anticoagulant therapy were more likely, whereas those with acute PVT, multiple thromboses or SIRS were less likely to survive.Conclusions and Clinical Importance: Hepatic disease is a common pre-existing condition in dogs with PVT. PVT should be considered in dogs with risk factors for venous thrombosis presenting with abdominal pain, ascites, and thrombocytopenia. Studies evaluating anticoagulant therapy in the management of PVT are warranted.