BACKGROUND Adenoid cystic carcinoma (ACC) is a malignant tumor that involves the salivary glands and is rarely seen in the nasopharynx. It is characterized by pain, a high rate of recurrence, and poor survival rate and poor prognosis as it tends to invade the perineurium. Due to the ambiguity of patient presentations, most patients present at a late stage, at which point metastasis has already occurred. CASE REPORT The patient in this case report was an otherwise healthy 56-year-old male smoker who presented to the clinic in March 2022 complaining of nasal obstruction, mouth breathing, recurrent epistaxis, headache, and facial pain. He reported significant weight loss in the past 6 months. The nasal scope showed nasopharyngeal fullness and biopsy-confirmed ACC exhibiting tubular, solid, and focal cribriform growth patterns. Further work-up showed an advanced form of nasopharyngeal ACC with intracranial extension and lung metastasis. The patient received concurrent radiotherapy and chemotherapy. After 6 months, the patient reported an improvement in the pain, and repeated images showed regression of the tumor size. CONCLUSIONS ACC is a malignant disease that is rarely seen in the nasopharynx. Currently, the most commonly agreed upon management for resectable ACCs in the literature is through surgical resection with adjuvant radiotherapy. However, most of the reported cases received radiation and chemotherapy due to the difficulty and risk of radical surgical excision of nasopharyngeal malignant tumors. Patients should be followed up to rule out any local recurrence or distant metastasis.