Aim: To measure leptin and homocysteine levels in serum of pediatric patients with portal hypertension, in order to assess their role in the development of hemodynamic and nutritional changes. Material and Method: Twenth-four patients with intrahepatic portal hypertension (IHPH), ten patients with extrahepatic portal hypertension (EHPH) and twenty healthy children as control group were evaluated by anthropometric measurements; height, height Z score, weight, weight Z score, weight-to-height, weight-to-age, body mass index (BMI), mid-arm circumference (MAC) and triceps skinfold thickness (TSF). Serum levels of leptin, homocystein, B12 vitamin and folic acid in all cases and serum levels of homocysteine in patients after methionin loading were measured. B12 and folic acid levels were measured by RIA, homocystein and leptin levels were measured by ELISA. Results: No statistically significant difference was found between the three groups according to sex, folic acid and B12 vitamin levels, but there was statistically significant difference in measurements of height, height Z score, weight, weight Z score, weight-toheight, weight-to-age, BMI, MAC, TSF, leptin and fasting homocysteine levels between the control and the IHPH groups. Between the control and the EHPH groups there was only significant difference in measurements of leptin and fasting homocysteine levels. No statistically significant difference was found between IHPH and EHPH according to levels of parameters. Conclusions: As a result, we observed malnutrition very frequently in both EHPH and IHPH groups, and IHPH group was more severely affected according to nutritional values. Serum homocysteine and leptin levels didn't significantly differ in between IHPH and EHPH groups. These results suggest that homocysteine which plays an important role in the etiology of atherosclerosis, doesn't play the same role in the etiology of portal hypertension.