Lipoblastoma is extremely rare and mainly occurs in children younger than 3 years old. It is predominantly found in the extremities and trunk. Head and neck region occurrences are rare; only 4 such cases involving patients who presented with stridor have been previously reported. We report the fifth case of lipoblastoma of the neck with stridor in a 9-year-old girl, which had gradually worsened over the previous year. Imaging showed a retrotracheal mass extending superiorly to the thyroid level and inferiorly to below the carina of the trachea. Total resection of the tumor was performed, and the histopathologic findings were consistent with lipoblastoma. Postoperatively, the patient was well with no complications.