With the deeper understanding of the pathophysiological mechanism of portal hypertension and the enrichment and refinement of examination methods, special portosystemic collateral veins (PSCVs) caused by portal hypertension are constantly identified and recognized. However, early identification and diagnosis and formulation of reasonable and effective treatment regimens are difficult issues in clinical practice due to complicated clinical manifestations, difficulties in diagnosis and treatment, severe complications, poor prognosis, and high mortality. This article elaborates on the anatomic structure, clinical significance, diagnostic methods, and treatment of special PSCVs caused by portal hypertension.