Introduction: The term lipoblastoma was introduced by Jaffe in 1926. It is a benign mesenchymal neoplasm of uncommon embryonic adipose tissue, made up of adipocytes in different stages of maturation, 90% of cases occurring in patients younger than 3 years. Case report: The case of a 2-year-old patient with a tumor in the upper third of the right thigh is described, with poor adherence to medical surveillance, performing definitive treatment at 6 years of age with a histopathological diagnosis of lipoblastoma. Discussion: Lipoblastoma occurs most frequently in extremities and trunk, progressive growth; magnetic resonance is the most sensitive radiological study to make diagnosis, and treatment is surgical. Conclusion: Lipoblastoma is a benign neoplasm of soft, mesenchymal parts, which occurs predominantly in childhood; there is a risk of recurrence in both the localized and the diffuse form, consequently surveillance is desirable for 5 years after resection. Our patient is without recurrence and with good quality of life.

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