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Idiopathic noncirrhotic portal hypertension is a heterogeneous group of diseases characterized by portal hypertension in the absence of cirrhosis. The efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) in this population are unknown. The charts of patients with idiopathic noncirrhotic portal hypertension undergoing TIPS in seven centers between 2000 and 2014 were retrospectively reviewed. Forty-one patients were included. Indications for TIPS were recurrent variceal bleeding (n 5 25) and refractory ascites (n 5 16). Patients were categorized according to the presence (n 5 27) or absence (n 5 14) of significant extrahepatic comorbidities. Associated conditions were hematologic, prothrombotic, neoplastic, immune, and exposure to toxins. During follow-up (mean 27 6 29 months), variceal rebleeding occurred in 7/25 (28%), including three with early thrombosis of the stent. Post-TIPS overt hepatic encephalopathy was present in 14 patients (34%). Eleven patients died, five due the liver disease or complications of the procedure and six because of the associated comorbidities. The procedure was complicated by hemoperitoneum in four patients (10%), which was fatal in one case. Serum creatinine (P 5 0.005), ascites as indication for TIPS (P 5 0.04), and the presence of significant comorbidities (P 5 0.01) at the time of the procedure were associated with death. Mortality was higher in patients with significant comorbidities and creatinine 100 lmol/L (P < 0.001). Conclusion: In patients with idiopathic noncirrhotic portal hypertension who have normal kidney function or do not have severe extrahepatic conditions, TIPS is an excellent option to treat severe complications of portal hypertension. (HEPATOLOGY 2016;64:224-231) I diopathic noncirrhotic portal hypertension (INCPH) is a heterogeneous group of rare diseases characterized by portal hypertension (PHT) without cirrhotic changes, without a cause of chronic liver disease, and without venous obstruction.(1,2) Various histologic changes may be present, including nodular regenerative hyperplasia, hepatoportal sclerosis or obliterative portal venopathy, sinusoidal dilatation, and Abbreviations: HE, hepatic encephalopathy; INCPH, idiopathic noncirrhotic portal hypertension; PHT, portal hypertension; PVT, portal vein thrombosis; TIPS, transjugular intrahepatic portosystemic shunt.