Choanal atresia (CA) is an obliteration or blockage of the posterior nasal aperture, limiting or obstructing the nasal airway to the nasopharynx. The presentation of unilateral CA commonly occurs at a later age than those children affected by bilateral CA (BCA) and can often persist undiagnosed into adulthood. BCA, on the other hand, typically presents with respiratory obstruction within the first few days of life, or desaturations with or without cyanosis when feeding or during exertion and traditional teaching is that this is a life-threatening emergency. We present the case of a 5-year-old girl referred to our department with nasal obstruction, snoring and some mild rhinorrhoea. After investigation she was found to have a mixed bony and membranous BCA and no other craniofacial abnormalities. The BCA was subsequently repaired using urethral dilators and a drill and the child is currently asymptomatic of any restenosis. This case prompts discussion of the various presentations and options in management of CA as well as allowing us an opportunity to discuss the literature on the subject.