Introduction: Congenital salivary gland anlage tumour of the nasopharynx is a lesion which usually presents with nasal and upper respiratory tract obstruction in the neonatal period. Timely diagnosis is essential to prevent the occurrence of respiratory complications in later childhood. Case Report: We present a 8-year-old boy complaining from difficulty in breathing and breastfeeding in the neonatal period due to an adenoid-like nasopharyngeal mass. Histological examination revealed solid and cystic squamous nests and numerous duct-like structures within collagenised stroma. Both epithelial and myoepithelial differentiation were noted in the tubular component. Discussion: A review of the clinical and histopathological features of published cases revealed that ancient lesions showed more prominent and complex epithelial component and more collagen rich stroma. We would like to suggest the possibility of salivary gland anlage tumour to be considered in the differential diagnosis of neonatal respiratory distress cases.