Purpose <p>Adenoid cystic carcinoma (ACC) of the submandibular gland is a rare malignancy characterized by an indolent clinical course and a high propensity for distant metastases. Hepatic metastases are particularly uncommon, and evidence guiding their optimal management is limited. This report aims to describe a rare case of isolated liver metastasis from submandibular gland ACC treated surgically and to contextualize it within the existing literature.</p> Methods <p>We present the case of a 58-year-old woman who developed a solitary hepatic metastasis four years after primary surgical resection and adjuvant chemoradiotherapy for submandibular gland ACC. A multidisciplinary treatment strategy was adopted, including liver venous deprivation (LVD) to optimize the future liver remnant, followed by right hepatectomy. In addition, a comprehensive review of the literature was performed to identify all reported cases of surgically treated hepatic metastases from submandibular gland ACC.</p> Results <p>The patient underwent successful right hepatectomy without major postoperative complications. Histopathological examination confirmed metastatic ACC with immunohistochemical features consistent with the primary tumor. At one year of postoperative follow-up, the patient remained disease-free. The literature review revealed marked heterogeneity in timing of metastasis, surgical approaches, and oncological outcomes, with variable disease-free survival and recurrence patterns following hepatic resection.</p> Conclusion <p>Surgical resection of isolated hepatic metastasis from submandibular gland ACC appears feasible and may provide durable disease control in carefully selected patients. A multidisciplinary approach, including preoperative liver hypertrophy techniques, can safely expand surgical indications. Given the potential for late metastatic recurrence, individualized treatment strategies and long-term follow-up are essential.</p>