
Odontogenic myxoma (OM) is a rare and locally invasive benign neoplasm found exclusively in the jaws. It commonly occur in the second and third decade. The mandible is more involved than maxilla. The lesion often grows without symptoms and presents as a painless swelling. The radiographic features are variable, and the diagnosis is therefore not easy. Radiographically myxomas appear as unilocular or multilocular radiolucencies. The radiolucent defect can contain thin, wispy trabeculae of residual bone which are often arranged at right angles to each other. The Histopathology of myxomas shows loosely arranged spindle-shaped stellate cells and few areas of inactive odontogenic epithelium in a mucoid intercellular substance. OM tumor cells are mesenchymal in origin and express vimentin and muscle- specific actin. In OM, Enucleation and curettage has proved an effective approach. This paper highlight a case of 13 years old male of OM in the maxilla and short review of the current literature on its pathogenesis, radiographic features, histopathology and surgical management.