Humans have the ability to nonverbally represent the approximate numerosity of sets of objects. The cognitive system that supports this ability, often referred to as the approximate number system (ANS), is present in early infancy and continues to develop in precision over the life span. It has been proposed that the ANS forms a foundation for uniquely human symbolic number and mathematics learning. Recent work has brought two types of evidence to bear on the relationship between the ANS and human mathematics: correlational studies showing individual differences in approximate numerical abilities correlate with individual differences in mathematics achievement and experimental studies showing enhancing effects of nonsymbolic approximate numerical training on exact, symbolic mathematical abilities. From this work, at least two accounts can be derived from these empirical data. It may be the case that the ANS and mathematics are related because the cognitive and brain processes responsible for representing numerical quantity in each format overlap, the Representational Overlap Hypothesis, or because of commonalities in the cognitive operations involved in mentally manipulating the representations of each format, the Operational Overlap hypothesis. The two hypotheses make distinct predictions for future work to test.