AbstractBackgroundDysgenetic polycystic salivary gland disease is a rare pathology. No case of minor salivary gland involvement has been reported in the literature.MethodsA female patient presented with bilateral tumors of the parotid glands, bilateral submandibular gland enlargement, and multiple cystic lesions of the oral mucosa. MRI of the neck was performed along with fine‐needle aspiration (FNA) of the parotid tumors and excisional biopsy of an oral lesion.ResultsImaging and FNA findings of the parotid glands were suggestive of bilateral Warthin tumors. Excisional biopsy of the oral lesion revealed a minor salivary gland with histopathological features of dysgenetic polycystic salivary gland disease. Submandibular gland enlargement was also attributed to this rare entity based on the imaging characteristics.ConclusionThis is the first report of dysgenetic polycystic salivary gland disease of the minor salivary glands, combined with involvement of the submandibular glands and bilateral Warthin tumors of the parotid gland. © 2016 Wiley Periodicals, Inc. Head Neck 38: E2437–E2439, 2016