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Lipoblastoma is a rare benign soft tissue tumor that occurs most commonly in infants and children. However, retroperitoneal lipoblastomas are rare, occurring in <5% of cases. We report a case of large retroperitoneal lipoblastoma and the largest collection of known retroperitoneal lipoblastomas in children in the literature.A 3-year-old girl presented with left abdominal mass. Magnetic resonance imaging (MRI) revealed a soft tissue mass measuring 12 × 8 × 6 cm in the retroperitoneal region. The mass had a clearly defined margin and a reticular pattern with an interposing fat component. Based on these findings, the mass was suspected to be a soft-tissue tumor, most likely lipoblastoma.Laparotomy with resection of the retroperitoneal mass was performed. The tumor was easily dissected from the retroperitoneal space without injury to surrounding structure.A histopathological examination demonstrated the mature proliferation of adipocytes and spindle-shaped cells separated by fibrovascular septa accompanied by myxoid changes. The cells were separated into lobules by septa, and areas of immature adipocytes showing a signet-ring or multivacuolar appearance were present at the periphery. Histopathological diagnosis was lipoblastoma. Follow-up at 6 months revealed no evidence of recurrence.Retroperitoneal lipoblastoma is rare and tends to be large in size when diagnosed at presentation. Complete resection should not be delayed, as impingement on the surrounding structures is imminent.