Lipoblastomas are rare soft tissue tumors that predominantly affect the pediatric population. We describe a lipoblastoma of the right hand in a 16-month-old boy. Radiographically the tumor appeared large but fairly well circumscribed and composed primarily of fat. Pathologic evaluation revealed variably sized lobules of adipose tissue and myxoid immature mesenchymal tissue separated by prominent fibrous trabeculae. Cytogenetic analysis showed a clonal chromosomal rearrangement with a breakpoint involving chromosome 8q11.2, confirming the diagnosis of lipoblastoma and thus helping to expand the clinicopathologic spectrum of tumors in this diagnostic category.