A theory has emerged, suggesting that abnormalities in the auditory system may be associated with sudden infant death syndrome (SIDS). However, current clinical evidence has never been systematically reviewed. A systematic review was conducted according to the guideline of Preferred Reporting Items for Systematic Reviews and Meta-Analyses. PubMed, Embase and Web of Science were systematically searched through 7 September 2020. Only human studies with a reference group were included. Studies were eligible for inclusion if they examined infants exposed to otoacoustic emissions (OAEs), auditory brainstem response (ABR) or had autopsies with brainstem histology of the auditory system. SIDS was the primary outcome, while the secondary outcome was near-miss sudden infant death syndrome episodes. Two independent reviewers extracted data and assessed risk of bias, and the quality of evidence. Due to high heterogeneity, a narrative synthesis was conducted. Risk of bias and quality of evidence was assessed using the Newcastle-Ottawa Scale and Grading of Recommendations Assessment, Development and Evaluation. Twelve case-control studies were included. Seven studies on OAEs or ABR had a high degree of inconsistency. Contrarily, four out of five studies reporting on brainstem histology found that auditory brainstem abnormalities were more prevalent in SIDS cases than in controls. However, the quality of evidence across all studies was very low. This systematic review found no clear association between auditory system pathology and SIDS. The higher prevalence of histological abnormalities in the auditory system of SIDS may indicate an association. However, further studies of higher quality and larger study populations are needed to determine whether these findings are valid. CRD42020208045.