A 15-month-old female child presented with progressive right-sided facial swelling, nasal obstruction, and deviation of the nose to the left. Computed tomography revealed a cystic mass in the maxillary sinus with disruption of the medial orbital floor. Incisional biopsy of this mass was nondiagnostic and the mass continued to grow rapidly. The mass was removed by excisional biopsy and curettage with conservative margins via combined lateral rhinotomy and sublabial approaches. Intraoperatively, a large defect of the floor that extended to the orbital apex was noted. Histopathology revealed an odontogenic myxoma. Odontogenic myxomas are uncommon tumors that are usually seen in adults. Our case is unique because to the best of our knowledge, it is the first reported case with orbital involvement in the pediatric population.