This article aims to review and systematize prior works that investigate how the presence of women in top echelon positions of firms affects sustainability and to create an agenda to guide future research in this promising area. In contrast to previous reviews, ours examines how women in top echelon positions affect sustainability by distinguishing both the position women held in the firm (i.e. Board of Directors—BoD, top management team, CEO and relevant committees) and the specific elements of sustainability (i.e. activity, performance, and disclosure). Our structured systematic review resulted in 187 publications retrieved from Web of Science and Scopus and revealed that the presence of women in top echelon positions is associated with greater engagement in social and environmental projects. Their presence also positively influences the environmental and social performance and increases the level, quality, and transparency of sustainability disclosure. Furthermore, the presence of women in top echelon positions and the implementation of sustainable activities improve both the firm financial performance and value. However, conflicting results have also emerged. On the basis of these findings, research gaps and future research agenda are identified and presented.