Reports of congenital anomalies of the Eustachian tube are scare. These anomalies are usually associated with chromosomal abnormalities, most often in oculoauriculovertebral spectrum. We present a case of completely bony widened Eustachian tube, entering the cells of sphenoid sinus lateral recess. No wall defect between the sphenoid sinus and the tube was found, nevertheless the tube and middle ear had normal pneumatisation. The anatomy of the outer ear, otoscopy and hearing thresholds were normal on the ipsilateral side. At the same time, there were microtia, atresia of the external auditory canal, underdeveloped tympanic cavity and cochlea hypoplasia and deafness on the contralateral side, although the majority of previous published cases described ipsilateral temporal bone anomalies. The patient had no facial asymmetry and was not given a syndrome diagnosis.