Among all benign and reactive mesenchymal lesions, nodular fasciitis is most frequently misdiagnosed as a sarcoma due to its concerning clinical and histopathological features: it arises on fascial surfaces, grows rapidly, and may infiltrate into adjacent tissues and blood vessels. The most commonly involved sites include upper extremities, trunk, head, and neck, with rare occurrences in the oral cavity. Recurrences following both incomplete and complete excision are rare, and its natural history progresses through self-limited growth followed by spontaneous regression. Herein we present a rapidly growing nodular fasciitis of the buccal mucosa confirmed by molecular testing for USP6 gene rearrangements which recurred 3 weeks following complete surgical excision. Additionally, we review reports of oral cavity nodular fasciitis between 2011 and 2023. Increasing awareness and diagnostic accuracy of this benign mimicker of oral cavity malignancy will help prevent unnecessary radical surgical resections.
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