Introduction: Myxoma is a benign, but locally aggressive neoplasm that rarely appears in the skeleton. It usually occurs in 2nd and 4th decades of life, common in females, in mandibular premolar-molar region, spreading within marrow leading to expansion of cortex. The case in point shows a striking contrast as the myxoma occurred in a male, in the anterior maxilla & perforated the cortex instead of expansion. Objective: To diagnose and surgically excise the maxillary myxoma along with esthetic restoration. Materials and Method: The localized gingival enlargement of the anterior maxilla was diagnosed as myxoma from clinical, radiographical and histopathological examinations. The lesion was surgically excised; defect was curetted and grafted with Hydroxyapatite. The lip pedicle flap was used to cover the defect. The restoration was given after complete healing. Results: The wound healing was excellent. The lip flap was well accepted. The prosthetic restoration reestablished the esthetics of the patient. Discussion: This case presentation exemplifies the importance of clinical suspicion, diagnostic evaluation and management including reconstruction issues in this unique case of maxillary myxoma.Conclusion: Every gingival enlargement should be viewed with a high degree of clinical suspicion for early diagnosis of an aggressive lesion beneath an indolent looking swelling. This may lead to offering the patient a conservative surgery with esthetic restoration rather than disfigurement due to an extensive surgery later. Keywords: rare lesion, maxillary myxoma, gelatinous mass, conservative excision, lip flap, esthetic restoration