Congenital microtia-atresia (CMA) often involves the auricle, external auditory canal (EAC), tympanic cavity and ossicular chain but rarely involves the Eustachian tube (ET). 1,2 The ET, part of the middle ear, connects the nasopharynx and tympanic cavity. It extends from the anterior wall of the middle ear to the lateral wall of the nasopharynx and serves the functions of middle ear drainage, balancing air pressure and preventing retrograde infection from the nasopharynx. 3 ET dysfunction has been reported frequently and is associated with some diseases, but there are few reports on ET malformations. 2 Most ET malformations occur in the cartilaginous portion, and cases of abnormal enlargement of the bony portion are rare. 4 The few cases reported mainly describe imaging manifestations of enlarged bony ET and associated syndromes; however, no detailed description of any additional clinical features or imaging characteristics of other structures has been reported. 2,[4][5][6] Middle ear diseases, such as cholesteatoma, are more likely to extend to the sphenoid sinus through the enlarged ET. 2 Bacteria and viruses in the nasal cavity or paranasal sinus diseases, such as chronic sinusitis, are also more likely to enter the middle ear through the ET, forming otitis media. 4 Greater care must be taken to avoid damaging the enlarged ET during surgery of the nasal cavity, paranasal sinus and nasopharynx. 6 It is clinically critical to pay attention to the bony enlargement of ET.Inadvertently, our clinical experience revealed some patients with CMA with abnormally enlarged bony portions of the ET on temporal bone high-resolution computed tomography (HRCT). Therefore, we collected and analysed clinical and radiological data for nine patients (14 ears) with enlarged bony ET, representing the largest series of reported cases, currently. The objective of our study is to investigate clinical and radiological features in congenitally enlarged bony portions of the ET and provide references for diagnosis and treatment.