Congenital aplasia of the major salivary glands is rare, and there have been few cases of the condition reported to date. In many cases, absence of the salivary glands is associated with syndromes involving the ectodermal tissues. The xerostomia encountered in affected children is usually associated with increased risk for caries and infections of the soft tissues. The purpose of this paper was to describe the case of a 10-year-old boy with bilateral aplasia of the submandibular and parotid salivary glands and the preventive and restorative treatment rendered. This case study demonstrates the importance of timely diagnosis of this condition in order to prevent the serious oral complications from xerostomia.

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