Undifferentiated embryonal sarcoma of liver (USL) is a rare pediatric cancer. It typically occurs in the liver of children and young adults. Most patients present it at stage when surgical resection is no longer possible and face a dismal prognosis. Multimodality treatment consisting of combinational systemic chemotherapy and surgery may improve the prognosis. We report a case of a young aged girl with advanced USL who is successfully treated with three times of surgical intervention and combination chemotherapy. Patient remains free of recurrence 49 months after diagnosis.