Purpose of Review <p>Idiopathic Non-cirrhotic portal hypertension (INCPH) is an important cause of portal hypertension, comprising a range of disorders. Although the presentation is often similar, the underlying causes differ, and the exact pathophysiology of INCPH remains uncertain. Additionally, various terms are used by researchers globally to describe this condition. This review discusses recent advances in understanding the fundamentals, clinical features, etiopathogenesis, and treatment of INCPH over the past few years.</p> Recent Findings <p>INCPH accounts for about 10% of portal hypertension cases presenting with a variceal bleed. The incidence is slowly declining, possibly due to improvements in living standards. Clinically, INCPH may closely mimic cirrhosis in a young individual, with variceal bleeding, splenomegaly and anemia being the most common presentations. Near normal tests of liver functions, with normal or mildly elevated liver stiffness (&lt; 10 Kpa) combined with very high spleen stiffness measured by transient elastography aid in differentiating INCPH from cirrhosis. Exclusion of cirrhosis by a liver biopsy and normal or mildly elevated hepatic venous pressure gradient (HVPG) are however, required for a definitive diagnosis. Portal vein thrombosis may develop over time in a small proportion of patients, with unfavourable outcomes. If the variceal bleeding is managed well with endotherapy or shunt surgery, ten-year survival is very good in INCPH patients.</p> Summary <p>Considering the possibility of INCPH in relevant clinical scenarios is crucial. Hemodynamic and histological parameters are essential to establish the diagnosis and define the clinical outcomes. A global patient registry can help enhance our understanding of INCPH disorders.</p>