Lipoblastoma is an uncommon benign soft tissue tumor of infancy and early childhood with a predilection for the extremities. CT and MRI can confirm the presence of fat components in the tumor. In addition, MRI better shows the anatomical extent. By showing lipoblastoma proliferation, histology confirms the diagnosis. Gross total excision is the treatment of choice. The authors report a case of unusual lipoblastoma of the axillary region, composed of fatty components with multiple cystic areas presenting at birth, with recurrence 9 months after excision.