Abstract
This case report details the exceptionally rare co-occurrence of submandibular gland lithiasis and carcinoma ex-pleomorphic adenoma (CXPA), representing only the fourth documented instance worldwide. The patient, a 79-year-old male with a 10-year history of recurrent submandibular symptoms, was found to have both ductal and intraglandular stones alongside non-uniform glandular enlargement. Post-surgical analysis confirmed CXPA. The patient remained disease-free with good functional outcomes at 24-month follow-up. This case provides valuable insights, suggesting a potential pathophysiological link between the chronic inflammation associated with long-standing lithiasis and the malignant transformation leading to CXPA. It underscores the importance of comprehensive diagnostic evaluation for chronic or atypical submandibular gland conditions to rule out concurrent malignancy.