Purpose The purpose of this systematic review is to identify and evaluate the available scientific evidence on the effectiveness of rehabilitation approaches proposed to children with severe-to-profound prelinguistic deafness on the hearing, speech, and language skills development. Method Databases (PubMed, CINHAL, PsycInfo, Cochrane, ERIC, and EMBASE) were searched with relevant key words (children, deafness, rehabilitation approach, auditory, speech, and language). Studies published between 2000 and 2017 were included. The methodological quality of the studies was evaluated with the Quality Assessment Tool for Quantitative Studies, and the level of evidence was evaluated with the Oxford Centre for Evidence-Based Medicine Levels of Evidence. Every step of the selection and analysis was made by 2 independent judges. Results Of 1,739 articles listed in different databases, 38 met the inclusion criteria and were selected for analysis. The majority of included articles present a relatively low level of evidence. Rehabilitation approaches that do not include signs appear more frequently associated with a better auditory, speech, and language development, except for receptive language, than approaches that included any form of signs. Conclusion More robust studies are needed to decide on the approach to prioritize with severe-to-profound deaf children.