Lipoblastomatous tumors are quite rare neoplasms derived from embryonic white fatty tissues. The majority is seen in infancy and early childhood. Generally they arise in extremities. Here, we present a 12-month-old female infant presenting with a mediastinal and cervical mass, showing left diaphragmatic eventration. We reviewed these cases and discussed clinical manifestations, radiologic, and histopathologic findings and treatment of these tumors.