The Food and Drug Administration (FDA) has recently raised concern regarding the safety of cochlear implantation in the setting of programmable cerebrospinal fluid shunts. The purpose of this study was to evaluate the outcomes and complications of cochlear implantation in children who have a cerebrospinal fluid shunt. Retrospective chart review. Tertiary academic referral center. Twenty pediatric subjects with a cerebrospinal fluid shunt and cochlear implant (CI). Cochlear implantation in the setting of a cerebrospinal fluid shunt. Primary outcome measures included descriptive data regarding age at implantation, etiology of hearing loss, medical management, complications, and speech perception outcomes. The average age of CI candidacy was 30 months with an average 21.5 months delay to implantation. In 45% of cases the laterality of the shunt determined the ear to be implanted. Three of the subjects required a surgical intervention on the shunt before cochlear implantation. Three subjects had a concurrently programmable shunt and activated CI. Two of the three subjects had no complications as a result of the two devices; however, the third subject had significant interactions requiring multiple revision surgeries. For those with the cognitive ability to perform open set, recoded speech perception, the average postoperative Consonant Nucleus Consonant word score in the best aided condition was 65.2% (n = 5). Children with a cerebrospinal fluid shunt are viable candidates for cochlear implantation, although they often require additional procedures and considerations before and after implantation.