BACKGROUND. Wildervanck syndrome was described for the first time in 1952. This rare entity is composed by the oculo-cervico-acoustic triad, which may be more or less complete. MATERIAL AND METHODS. We present a 5-year-old girl diagnosed with Wildervanck syndrome, exhibiting: Klippel-Feil anomaly (fused cervical vertebrae), Duane Stilling Turk syndrome (bilateral abducens palsy) and congenital bilateral cophosis indicated by ABR. RESULTS. The CT scan revealed major malformations of the brain and calvarium including the petrous part of both temporal bones. Despite all these brain malformations, the psychological development of the child was nearly normal. As it was previously reported in other cases, the child also presented spina bifida cervicalis and cleft palate. The cause of deafness was bilateral lack of cochleae, making impossible the insertion of the cochlear electrode for hearing rehabilitation. When the patients are discovered late (after 3 years of age), the management of hearing loss is by being taught sign language and lip reading. CONCLUSION. Any cranio-facial malformation and any delay in speech production impose early attention of the otorhinolaryngologist or audiologist for timely diagnosis and rehabilitation of hearing loss. The imaging examination, especially the high-resolution temporal bone CT-scan, is of paramount importance to be performed in all profound congenital deaf children to evaluate the candidacy to cochlear implantation.