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The authors present the clinical picture, histopathologic findings and surgical treatment of an infant born with a lipoblastoma involving an unusual combined orbito-nasal location. To the best of our knowledge, this is the first report of congenital lipoblastoma extension from orbit to the nasal cavity. Transconjunctival orbitotomy with excisional biopsy of tumor was performed. Histology specimen that demonstrates the small embryonal fat cells in different stages of maturation and the characteristic lobulation of the fat tissue are presented. The embryonic origin, timing of development of the tumor during gestation, as well as the route of propagation from orbit to nose are discussed. The authors conclude that complete excision of this tumor, with preservation of normal tissue, may bring cure to the young patient without gross functional deformity.