Granular cell tumors are rare tumors with multiple localizations. The most common localizations are in the cervicofacial region in 45–65% of cases. The characterization of this tumor depends on its clinical and histopathological findings. A few cases have been reported describing more unusual localizations, such as in the juxta-axilla or in the gastrointestinal tract. In this article, we describe an original localization: the region of the temporomandibular joint. This case-report describes a clinical case that was managed in the Department of Maxillofacial and Oral Surgery of the Saint-Joseph Hospital. The tumoral lesions usually described for this anatomical location are usually bony or synovial lesions. Here, the clinical examination didn't correspond to any of those descriptions and the MRI also confirmed that this tumor characteristics weren't compatible with this kind of lesion.