Background: Lipoblastoma is a rare, benign, fatty tissue tumor that occurs in infancy and early childhood. The most common tumor locations are the extremities and the torso. The location of this tumor in the chest wall and an intrathoracic extension is uncommon.Case report: We present a case of a 3-year-old boy with anterior chest wall lipoblastoma with an intrathoracic extension. Computed tomography was suggestive of lipoblastoma. The mass was completely excised through a right posterolateral thoracotomy. The histologic examination of the lesion confirmed the diagnosis of lipoblastoma.Conclusion: Although extremely rare, chest wall lipoblastoma should be included in the differential diagnosis of thoracic mass in childhood.