Oral focal mucinosis (OFM) is a rare connective tissue disorder characterized by myxoid degeneration of submucosal connective tissue. It usually presents as gingival or mucosal overgrowth. Due to its uncommon occurrence and lack of pathognomonic clinical or radiological features, diagnosis mainly relies on histopathological evaluation. The paper reports a rare case of large OFM in a 58-year-old female patient involving the posterior maxilla and hard palate. Diagnosis of the lesion was established based on histopathological and immunohistochemical analysis. The lesion was excised surgically and showed no recurrence at 1 year follow-up. The cases presented intend to bring OFM to the attention of oral pathologists and clinicians while considering the differential diagnosis of myxoid lesions of oral cavity.