Hepatoportal sclerosis, now categorized under the term porto-sinusoidal vascular disease, is a condition leading to non-cirrhotic portal hypertension and posing a substantial risk of variceal bleeding. This payer aims to elucidate this phenomenon through a case study involving a patient displaying manifestations of non-cirrhotic portal hypertension with hemorrhagic indications. The objective is to describe the clinical, radiological, and histological features, as well as the complications associated with this pathology, which is infrequently documented in the pediatric population.