INTRODUCTION: Lipoblastoma is a rare benign tumor arising from embryonic white fat which occurs in the early childhood. It usually arises on the extremities and considered as a rare cause of a pediatric head and neck masses. The aim of this study is to shed light on lipoblastomas as a differential diagnosis of rapidly growing soft fatty masses of children in neck and head area. PATIENTS AND METHODS: A retrospective review of 3 patients with lipoblastoma, underwent Surgical resection (case 1 and 2) by cervical approach. The third patient with a facial lipoblastoma was not operated due to the high risk of facial paralysis. Review of literature, diagnostic methods and genetics of lipomatous tumors are discussed. RESULTS: Complete surgical excision via a cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology analysis confirmed the diagnosis of lipoblastoma. DISCUSSION: Lipoblastoma is a rare childhood tumor, even rarer in head and neck area. The pathogenesis is unknown, though it is believed to arise from altered embryogenesis of human white fat and genetic predisposition, as chromosome 8 abnormalities may be implicated in the development of lipoblastoma. The presumptive diagnosis is performed by imaging. The most important differential diagnosis of lipoblastoma is myxoid liposarcoma. The mainstay of treatment is complete non-mutilating resection of the tumor to avoid recurrence. CONCLUSION: Lipoblastoma should be suspected in case of heterogeneous fatty tumor in head and neck area, and included as a differential diagnosis of cervical masses in children younger than 3 years. The mainstay of treatment is complete surgical excision with a good prognosis.