© 2020, Springer Science+Business Media, LLC, part of Springer Nature. Hyalinizing clear cell carcinoma (HCCC) is a rare, low-grade neoplasm accounting for approximately 1% of salivary gland neoplasms. Histologically, it is characterized by a monomorphous population of clear cells arranged in sheets, nests, or cords, lacking ductal structures. Until recently, clear cell carcinoma of the oral cavity (OC) represented a diagnosis of exclusion when other head and neck pathologic entities such as epithelial-myoepithelial carcinoma or mucoepidermoid carcinoma could be ruled out, making definitive diagnosis by light microscopy and immunoprofiling a challenge. As a result, initial biopsies are often misclassified, and could result in under- or overtreatment. More recently, the presence of the EWSR1-ATF1 gene fusion has been adopted to definitively diagnose HCCC. Typically, HCCC demonstrates clinical indolence and responds well to curative surgical excision alone for localized disease, with adjuvant radiotherapy (RT) reserved for high risk features including perineural invasion, lymphovascular invasion, and regional cervical metastasis. The literature, however, lacks consensus regarding the role of adjuvant radiotherapy. In this article, we report a case of HCCC in a rare site involving the ventral tongue, with high risk features of perineural invasion and cervical nodal metastasis. The patient underwent surgical excision alone; declining adjuvant radiotherapy despite the high risk features, and was alive with no evidence of disease at the 42-month mark. Furthermore, we provide an update on the current prognostic indicators for HCCC, and emphasize the need for chromosomal analysis to achieve a definitive diagnosis.