The estimated incidence of sensorineural hearing impairment (>40 dB HL) at birth is 1.86 per 1000 newborns in developed countries and 30-40% of these are unilateral. Profound sensorineural unilateral hearing impairment or single sided deafness (SSD) can be treated with a cochlear implant. However, this treatment is costly and invasive and unnecessary in the eyes of many. Very young children with SSD often do not exhibit language and cognitive delays and it is hard to imagine that neurocognitive skills will present difficulties with one good ear. In the current paper we review the most recent evidence on the consequences of unilateral hearing impairment for auditory and neurocognitive factors. While data of both adults and children are discussed, we focus on developmental factors, congenital deafness and a window of opportunity for intervention. We discuss which etiologies qualify for a cochlear implant and present our multi-center prospective study on cochlear implants in infants with one deaf ear. The large, state-of-the art body of research allows for evidence-based decisions regarding management of unilateral hearing loss in children.