Complete congenital agenesis of all major salivary glands: A case report and review of the literature

Congenital agenesis of the salivary glands is uncommon. There are documented cases of partial or unilateral aplasia of the major salivary glands associated with the lacrimal puncta, but very few reports of the absence of all major salivary glands. We report the case of a 10-year-old girl with xerostomia and extensive teeth caries. Physical examination and imaging showed total and bilateral aplasia of the parotid, submandibular, and sublingual glands, with no involvement of the minor salivary glands or the lacrimal puncta. We describe the clinical presentation, important aspects in diagnosing partial forms of the condition, and patient management.