Myxomas are benign, slow-growing, locally invasive neoplasms derived from mesenchymal elements. Infantile myxoma (odontogenic or sinonasal) is extremely rare and usually occur in children older than 10 years of age. A 18-month-old boy presented with a painless, enlarging elevated lesion in left paranasal area. Physical examination revealed a firm, non-tender, bony-like elevation in the left nasal bone- maxilla bone transition zone that extended to the level of the inferior orbital rim, adjacent to the nasal bone. A computed tomography scan of the head revealed a well-defined, hypodense, multilocular lesion with a peripheral hyperdense circumference, involving the anterior wall of the maxilla, from the pyriform aperture to the level of orbital floor, adjacent to the nasal bone. Surgery was performed using an external approach. The microscopic evaluation showed loosely arranged spindle cells spaced in a rich myxoid matrix. Although rare, sinonasal myxoma should be included in the differential diagnosis of slow-growing, well-defined lesions of the face and jaws in infants.