Childhood respiratory diseases, such as asthma, are important public health problems worldwide and could be associated with tooth enamel defects. This study aimed to verify the relationship between asthma and enamel defects in teeth, to answer the following question: "Could asthma in children be significantly associated with enamel defects in deciduous dentition and young permanent teeth?." PUBMED-MEDLINE, EMBASE, LILACS, and COCHRANE databases were systematically searched and assessed articles were cautiously scored according to a predetermined criterion. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses were considered. Twenty-two articles were critically appraised and used as a basis for conclusions. The relationship between asthma and enamel defects was confirmed in the majority of appraised papers, one with a high level of evidence, nine with a moderate level, and four with a low level. Out of the eight manuscripts investigating the influence of asthma medication on enamel defects, only three (one with high, one moderate, and another with a low level of evidence) suggested an association. It can be concluded that asthma is closely connected with enamel defects in young permanent teeth. However, as most of the papers appraised were of cross-sectional or case-control design, further welldesigned clinical investigations with a prospective design are welcome to reinforce our findings.