The authors describe the first reported case of spindle cell tumor of the portal vein (SCTPV) surgically excised in a 28-year-old woman. The tumor had caused portal vein obstruction with extensive collaterals. A side-to-side mesocaval shunt was created to reduce portal hypertension. Six months later the SCTPV could be excised. Radiation therapy (4,500 cGY) was given 2 months later. Although the tumor recurred in the liver 4 years later, the patient is alive and free of symptoms 6 years after the initial diagnosis.