Adenoid Cystic Carcinoma (ACC) is a malignant epithelial neoplasm originating in the salivary glands, marked by indolent growth, pronounced local recurrence, and neurotropic invasion, frequently exhibiting perineural spread and posing significant diagnostic challenges in differentiation from other salivary gland tumors. This report presents a case of a recurrent, low-grade sinonasal malignancy with a challenging pathological diagnosis, ultimately confirmed as ACC through fluorescence in situ hybridization (FISH). A comprehensive review of pertinent literature is included to outline the clinical and pathological features of sinonasal ACC and distinguish it from other sinonasal malignancies, aiming to enhance clinical recognition and facilitate diagnosis in complex cases.