BACKGROUND Adenoid cystic carcinoma (ACC) is a malignant tumor commonly occurring in the major salivary glands. ACC of the nasal septum is exceeding rare. METHODS The case of a 42-year-old woman with ACC of the nasal septum is presented. Her chief complaint was nasal obstruction and dysosmia for two month. CT and MRI demonstrated a massive mass occupying the nasal septum infiltrating the palate, vomeronasal, anterior skull base, and dura mater. Combined anterior cranial surgery, endoscopic intranasal surgery, and transpalatal surgery were selected due to the size and location of the tumor. A negative surgical margin was achieved without cosmetic deformity or functional disorder. RESULTS She had postoperative radiotherapy with no recurrence or distant metastasis during the follow-up period. CONCLUSIONS The tumor location and the perineural spread pattern should be considered to determine the treatment plan for septum ACC. Post operative radiation is now generally recommended. ACC has a high incidence of local recurrence and distant metastasis rate; therefore, long-term follow-up is necessary.