A salivary gland anlage tumour (SGAT) is a very rare type of benign tumour that usually presents in early infancy with respiratory distress which is exacerbated upon feeding. We report a full-term male neonate who was referred to the Al Nahdha Hospital, Muscat, Oman, in 2015 with severe neonatal respiratory distress due to a nasopharyngeal obstruction immediately after birth. Computed tomography and magnetic resonance imaging revealed a well-circumscribed mass in the nasopharynx, without intracranial extension. Histopathological analysis of the lesion confirmed a diagnosis of SGAT. Following excision of the tumour, the postoperative period was uneventful. No recurrence was observed over the next two years. This case report highlights the importance of the early recognition of this extremely rare and potentially life-threatening, yet easily curable, condition.