Early number skills, comprised of both informal and formal skills, are associated with later mathematics achievement. Thus, the development of foundational early number skills is an important aspect of early mathematics instruction. This study explored relations between early number skills gains and mathematics achievement for students at risk for mathematics difficulties in a kindergarten intervention study. Results indicated strong relationships between formal number skills gains and mathematics achievement across kindergarten and Grade 1. Intervention participants demonstrated larger informal and formal early number skills gains compared with their control peers, and relations between early number skills gains and first-grade mathematics achievement were moderated by intervention participation. Importantly, these findings suggest that formal and informal number skills gains may be critical components of later mathematics achievement for at-risk students. Implications for special education and intervention in tiered instructional systems are discussed.