Purpose: Non-cirrhotic portal fibrosis (NCPF), or Idiopathic Portal Hypertension, is an important cause of non cirrhotic portal hypertension leading to significant morbidity and mortality. It constitutes nearly 10–15% of all causes of variceal bleed in developing countries. We present the clinical profile of a large series of well characterized patients of NCPF. Methods: A retrospective analysis of all patients with NCPF consecutively seen since 1983 was done. The diagnosis of NCPF was based on the presence of varices, dilated, thickened and patent portal vein and normal liver histology. Their clinical records were retrieved and analyzed. Results: 366 patients were included in the study. The male:female ratio was 1:1, and 98% were from low socio-economic strata of society. The mean age of presentation was 31.6 (±13.8) years. 262/366 (72%) patients had presented with GI bleed. The frequency of bleeders increased with age: ≤12 yrs 50%, 13–24 yrs 66%, 25–36 yrs 72%, 37–48 yrs 73%, 49–60 yrs 78% and >60 yrs 93%. The bleeders had presented with median 1 episode (range 1 to 20) of bleeding prior to presentation. Those who had bled more than once, tolerated the bleeds well and had a median of 3 bleeding episodes in a median span of 6 months (bleeding risk 0.5 episodes/month).The bleeders had required a median of 2 units of blood transfusion for their bleed. 97% patients had esophageal varices with a median grade of 3. 31% had gastric varices, 70% of them GOV1. Median HVPG (N = 53) was 7 mmHg (range 2–15 mmHg), diagnostic of pre-sinusoidal site of resistance in NCPF. History of pain abdomen was found in 27%, transient ascites in 25%, jaundice in 18%, pedal edema in 18%, and lump left abdomen in 12%. Splenomegaly was seen in 74%, the mean spleen size was 8 (±5) cm below costal margin. 41% had Hb ≤ 7 gm/dl, 22% had TLC ≤ 4×103/mm3 and 27% had platelets ≤ 80×103/mm3. Liver function tests were normal in 98%, with median bilirubin of 0.9 mg/dl, AST 39 IU/L, ALT 35 IU/L, alkaline phosphatase 193 IU/L, albumin 3.7 g/dl and prothrombin time within 3 seconds of control. HBsAg was positive in 5.7% and anti-HCV in 2.2%, predominantly due to transfusions. The histology of liver showed normal hepatic architecture, mild kupffer cell hyperplasia and no evidence of chronic hepatitis or cirrhosis. Conclusions: Gastrointestinal hemorrhage is a serious complication of NCPF and it presents predominantly in the young adults. Though the rebleeding risk is high, the bleeds are well tolerated. The liver functions, liver architecture and HVPG remain normal in these patients.