Myxomas of the maxillofacial region are neoplastic entities of mesenchymal origin most often associated with odontogenic origin; sinonasal myxoma is rare, located in the nasolabial region and originating from the sinonasal tract. The aim of the current study was to report a well-documented case of sinonasal myxoma in a 12-month-old boy, initially presenting with obliteration of his left nasolacrimal duct. A soft-tissue mass of the nasobuccal groove, firmly attached to the underlying bone, was revealed. After biopsy where benign fibroblastic elements were found, the tumor was removed surgically in wide margins, whereas great care was taken to reconstruct the involved adjacent anatomic structures and preserve facial aesthetics. Histopathologic findings were compatible with an extragnathic, nonodontogenic sinonasal myxoma originating from the nasolacrimal duct. The clinical significance of the case presented was its rather rare location and origin. Three and a half years postoperatively, functional and aesthetic results were satisfactory with no sign of recurrence. To the authors' knowledge, this is the second youngest reported case in the literature.