Congenital bilateral parotid gland agenesis in Klinefelter syndrome

Bilateral parotid gland agenesis is a rare clinical entity and it's an etiopathogenesis remains obscure. The literature contains no reports of the co-existence of bilateral parotid gland agenesis and Klinefelter syndrome. The condition is usually asymptomatic and causes asymmetry in the head and neck areas. Its diagnosis is generally made in the light of accompanying developmental anomalies. Herein we report the clinical and radiological findings of a 17-year-old male with Klinefelter syndrome accompanied by unilateral peripheral facial nerve paralysis and isolated congenital bilateral parotid gland agenesis.