Adenoid cystic carcinoma is a malignant tumor with a deceptively benign histologic appearance characterized by indolent, locally invasive growth with high propensity for local recurrence and distant metastasis. The tumor is composed of basaloid cells with small, angulated, and hyperchromatic nuclei and scant cytoplasm arranged into 3 prognostically significant patterns: cribriform, tubular, and solid. Some tumors undergo dedifferentiation into a high-grade form. Numerous studies have attempted to elucidate accurate histologic prognostic features but have often yielded conflicting results. Microarray analysis and gene expression profiling have provided new potential diagnostic and prognostic markers. However, tumor grade, stage, lymph node metastasis, invasion of major nerves, and margin status remain the most consistent predictors of prognosis. The combination of surgery and postoperative radiation therapy has improved locoregional control of the disease. Despite this achievement, late local recurrence and distant metastasis rates remain high and may occur decades after initial diagnosis.