Research focusing on the educational strategies for students with severe/profound intellectual and multiple disabilities (S/PIMD) is strikingly scarce. Previous research has shown that the sensitive responsiveness of interaction partners, including school staff members, and motivating learning activities may promote interactive engagement (e.g., attention and initiation) in these learners. Interactive engagement is, in turn, considered important for learning and development more generally. Music interaction/therapy and multisensory storytelling are approaches that have shown positive results in supporting interactive engagement in individuals with S/PIMD. However, no known studies have investigated the effect of combining relevant elements from these approaches in educational settings with students with S/PIMD. The overall aim of the thesis was to develop new knowledge regarding how musical interaction with and without storytelling elements could support interactive engagement in students with S/PIMD using various methods in three sub-studies. Firstly, a scoping review of published peer-reviewed research was conducted to summarise the current evidence base on musical interaction with children and youths with S/PIMD (study 1). While the studies displayed great variation in quality and design, musical interaction was generally linked with positive outcomes for the participants with S/PIMD in several developmental domains, including communication, social interaction, engagement and cognition. Six categories of promising components of musical interaction were identified, namely the responsivity of the interaction partner, singing songs, structure and predictability in activities, a trusting alliance between interaction partners, long-term interventions, and using technology-mediated and multisensory music activities. Next, a novel pedagogical approach – MultiSensory Music Drama (MSMD) – was developed and empirically evaluated in terms of its effectiveness and social validity in school settings for students with S/PIMD (studies 2 and 3). To explore the effect of MSMD on interactive engagement, a study with a single-case-experimental design involving three participants was conducted (study 2). Data from the coded video observations showed higher levels of interactive engagement during MSMD relative to a control activity. Similarly, school staff members’ ratings of interactive engagement were higher for two of the students during MSMD. In the last study, a special education teacher and her student with S/PIMD working with MSMD were followed in a qualitative, longitudinal case study. The results showed how the teacher, in her day-to-day teaching, implemented and developed MSMD lessons, with the process summarised in the following categories: the reflective practitioner; enabling student agency; and the usefulness of MSMD. Conclusions and implications from this thesis point to the importance of finding motivating pedagogical activities to support interactive engagement in students with S/PIMD. This thesis shows that MSMD could be an effective activity that enhances interactive engagement in participants with S/PIMD. The findings from the thesis collectively show how theoretically motivated and concrete learning activities, focusing on musical interaction with and without storytelling, support interactive engagement for these students. Also, it is perceived as feasible and helpful to integrate these into the everyday activities in schools.