Angiomyxomas are a group of rare myxoid benign mesenchymal tumors prone to local recurrences. Two types of angiomyxomas are well recognized in the literature: superficial and aggressive angiomyxoma. Aggressive angiomyxomas are relatively uncommon, poorly circumscribed, locally infiltrative myofibroblastic tumors with a specific predilection for the perineal regions and exceedingly rare in the head and neck region. To the best of our knowledge, we report the first case of a gigantic aggressive angiomyxoma occurring in both the maxilla and mandible in a 30-year-old male patient. Clinicians should carefully evaluate the clinical, radiological and histological data to derive the correct diagnosis of this myxoid intraoral soft-tissue neoplasm.