The Every Student Succeeds Act (ESSA) requires 95% participation in state achievement testing. Students with the most significant cognitive disabilities participate in states' alternate assessments based on alternate academic achievement standards (AA-AAAS). A growing number of these students are also English learners (Christensen et al., 2018). This report summarizes an investigation of research published in 2000-2018 on evidence-based literacy assessment and instruction practices for English learners with the most significant cognitive disabilities. This report included research literature on students with intellectual disabilities, autism, and multiple disabilities because "significant cognitive disability" is not a disability category (as defined under the Individuals with Disabilities Education Act, or IDEA) and therefore there is dearth of research using the term. To locate articles addressing English learners with significant cognitive disabilities, the authors took a broad approach. In addition to students identified in a study as English learners, they included studies with any student in the included disability categories whose home language was not English. A two-phase search process for the literature review. In Phase 1, the reviewed articles included in a previous review conducted by Liu, Thurlow, and Quenemoen (2015) using a similar, but slightly narrower, set of inclusion criteria focused on peer-reviewed research and dissertations with students ages 6-21 years. In Phase 2, they conducted a three-part search for research published from 2015 to the end of 2018 using selected academic search engines, a hand search of key special education journals, and forward and reverse citation searches. Six of the eight articles from Phase 1 met the inclusion criteria, and eight studies met the inclusion criteria in Phase 2, for a total of 14 studies. [For the 2015 report, "Instructing and Assessing English Learners with Significant Cognitive Disabilities," see ED609760.]