ABSTRACT
Congenital absence of the major salivary glands, especially of the parotid gland, is a rare disorder whose etiopathogenesis is poorly understood. Aplasia of the parotid glands may be unilateral or bilateral and may occur alone or in association with the absence of other salivary glands or with other developmental anomalies of the first branchial arch, such as hypoplasia or aplasia of the lacrimal glands, hemifacial microsomia, mandibulofacial dysostoses, and multiple congenital anomalies. Various degree of xerostomia and dental caries with early loss of teeth may occur due to decreased salivary production. The authors experienced a case of unilateral parotid aplasia in a 22-year old female who had painless swelling in the right parotid region. We present this case with review of literature.