Although cochlear implantation has been almost a standard otological procedure worldwide, it may still create a dilemma for the surgeon in some unusual instances such as Seckel syndrome, the presence of a cardiac pacemaker for complete atrioventricular block, aural atresia, and in the presence of concurrent pathologies.Seckel syndrome is a rare constellation of malformations which is presumably inherited as an autosomal recessive trait. The primary characteristic features of Seckel syndrome are: intrauterine and postnatal dwarfism, severe microcephaly, orofacial dysmorphism with characteristic "Bird-headed" appearance, prominent beaked nose, micrognathia, variable mental retardation and the presence of other malformations, especially of the skeleton (1,2). Abnormalities associated with cardiovascular, hematopoietic, endocrine and central nervous systems were also reported in Seckel syndrome (3).Congenital aural atresia is a relatively common congenital anomaly in otology. It is estimated to occur in approximately 1 out of every 10,000 to 20,000 births. Hypoplasia of the external auditory canal, often associated with abnormalities of the auricle, middle ear and the inner ear structures, is the characteristic feature of aural atresia (4,5). It is unilateral in 70% to 85% of cases and the right ear is affected more frequently than the left (6). Intracranial arachnoid cysts are presumably congenital malformations that are believed to form by the splitting of the arachnoid membrane because of a circumscribed increased pulsation of the cerebrospinal fluid (7,8). Arachnoid cysts constitute approximately 1% of all intracranial lesions. The most common location of these lesions is the middle cranial fossa, with the posterior fossa being the second most common location. The clinical entities presented above are rare, and cochlear implantation in these entities is an extraordinary Background: Although cochlear implantation has been almost a standard otological procedure worldwide, it may still create a dilemma for the surgeon in some unusual instances such as Seckel syndrome, aural atresia and posterior fossa arachnoid cyst. Case Report: Three extraordinary cases of cochlear implantation were reported. The first case was a case of Seckel syndrome with a cardiac pacemaker due to complete atrioventricular block. The second case had posterior fossa arachnoid cyst that had retrosigmoid cyst removal and cochlear implantation simultaneously. The last case had cochlear implantation in the ear with congenital aural atresia. All cases could be implanted successfully with full electrode insertion and good audiological outcome. Delayed facial paralysis that occurred in the patient with arachnoid cyst resolved spontaneously. Conclusion: This study addressed the efficiency of cochlear implantation in cases of Seckel syndrome, complete atrioventricular block managed with cardiac pacemaker, congenital aural atresia and posterior fossa arachnoid cyst. In addition, the retrosigmoid approach and cochlear implantation can be performed simult...