Background: Only 8 cases of Rosai-Dorfman disease (RDD) involving the submandibular gland (SMG) have been reported. We report one more such case, involving a 51-year-old man, thus, adding to the available body of knowledge for this specific pathology. Methods: The patient presented with a growing mass in the right SMG. Along with physical examination, hematologic and imaging-based diagnostic techniques were used. A diagnosis of the right SMG tumor was made and, thus, resection of the right SMG along with the lesion was carried out. Results: Post-operative pathology was found to correspond to that of RDD. After 24 months of observation, the patient remained healthy. Conclusion: RDD involving the SMG is rare, but not improbable, and hence, should be considered during diagnosis, especially when accompanied by neck lymph node enlargement. This is imperative because RDD is generally benign and self-limiting, and thus, a correct diagnosis may prevent SMG resection.