Congenital absence of major salivary glands

Congenital absence of major salivary glands is infrequent, with only a few cases in the world medical literature. We found 30 documented cases in a bibliographic search. We report a case of total agenesia of the parotid gland diagnosed in the course of a study motivated by symptoms of facial pain and the temporomandibular joint. Agenesia of a major salivary gland (specially the partial ones) usually develops with no manifestation that motivates the patient to seek treatment. Parotid aplasia can be an isolated finding or associated with the absence of other glands or with abnormalities such as aplasia of the lacrimal gland, mandibulofacial dysostosis, and ectodermic dysplasia.