A case of major salivary gland agenesis.

Major salivary gland agenesis is a rare disorder and only a few instances of the condition have been reported. The number of absent salivary glands and the degree of associated xerostomia vary between individuals. Salivary gland agenesis can cause profound xerostomia in children and there may be oral and upper respiratory tract sequelae, such as dental caries, candidiasis, ascending sialadenitis, laryngitis and pharyngitis. Salivary gland aplasia may be associated with other ectodermal defects, particularly abnormalities of the lacrimal apparatus. We report on the first observation of major salivary gland agenesis in South Korea. A child with xerostomia should be appropriately investigated and diagnosed, and careful attention should be paid to the prevention and treatment of sequelae.