Adenoid cystic carcinoma (ACC) is a malignancy of the secretory epithelial cells of the salivary glands and constitutes less than 1% of all head and neck tumors. Metastasis occurs frequently and most commonly affects the lungs at a rate of 35 to 50%. In this case report, we present a rare case of nasopharyngeal ACC with distant metastasis to the liver. Our patient initially presented to the hospital with dental and sinus pain with initial imaging suggesting nasopharyngeal carcinoma invading the temporal lobe laterally and the cavernous sinus and clivus medially. The foramen ovale and the optic nerve were also involved, leading to loss of vision bilaterally. Immunohistochemical staining of the biopsy eventually led to the correct diagnosis of high-grade ACC, solid type. The patient’s hospital course was complicated, with pulmonary thrombosis eventually leading to hypoxic respiratory failure and death. Although this patient was initially diagnosed with nasopharyngeal carcinoma, thorough pathologic investigations allowed for a clearer understanding of the disease, primarily ACC’s eventual distal metastasis in the patient. In the future, providers should continue to keep ACC in their differential diagnosis list when evaluating patients with head and neck tumors, with the goal of maintaining locoregional control of the tumor.Keywords: adenoid cystic carcinoma, liver metastasis, nasopharyngeal salivary glands, head and neck cancer