Students with significant cognitive disabilities may participate in states' alternate assessments based on alternate achievement standards (AA-AAS). A growing number of students with significant cognitive disabilities are also English learners (ELs) whose family may use another language in the home. They may only be exposed to English in a school setting. To fully and meaningfully participate in an AA-AAS, ELs with significant cognitive disabilities need to be offered meaningful access to the grade-level curriculum prior to taking the assessment. The purpose of this article is to identify an evidence base on best practices in English language arts instruction for these students. In an effort to understand current practices for instructing ELs with significant cognitive disabilities, the authors examined the available literature on instruction for these students. From this review of literature they draw implications for creating curriculum and instruction materials to support the learning of those ELs who participate in an AA-AAS. Based upon the limited research available, the report provides recommendations for important first steps to ensure that ELs with significant cognitive disabilities have access to grade-level curriculum and instruction.