While studies of entrepreneurship and family business have to a great extent developed independently, there are some indications that they are now moving closer to each other. The purpose of this special issue is to contribute to an increased scholarly interest in research that integrates the areas of entrepreneurship and family business. This introductory article elaborates upon the meaning of entrepreneurial families and family firms. Based on a review of a significant amount of previous literature and the articles in this special issue, we generate a guiding framework around three themes - actor, activity and attitude. We argue that research focusing on specific topics within these themes has great potential to contribute to our theoretical and empirical understanding of entrepreneurship and family firms. We also share a note on why we believe Entrepreneurship and Regional Development is a suitable arena for publishing research with this orientation. We then introduce the five papers that are included in this special issue using the framework developed to position the papers and thereby to reveal their respective contributions and their advancement of our knowledge. We conclude with reflections on some unexplored themes, which are still very relevant to examine in future research on entrepreneurship and family businesses.