Salivary calculi occur most frequently in the submandibular gland of the human beings. It is because of the specific anatomy of both the glands and its duct. Presentation typically consists of a painful swelling of the gland at meal times, when the effect of obstruction is most acute. The clinical signs, in most of times, can lead us to the diagnosis easily. The coexistence of sialolith and malignant tumors are very rare. This case report is about a male patient of 45 year with a swelling in the submandibular region for the last eighteen months with an intermittent episode of moderate to severe pain which increased during a mealtime and diagnosed as sialolith on radiograph, but on biopsy proved to be adenoid cystic carcinoma along with sialolith. This case report describes the coexistence of sialolithiasis and adenoid cystic carcinoma in the submandibular gland with advantages and limitations of various imaging studies to be used by health care professionals prior to reaching definitive conclusion