Biotechnology is one of the strongest growing industries of the twenty-first century. Yet, the sector is still young and many biotechnology firms are at an early stage of their life cycle. Thus, biotechnology and entrepreneurship are intrinsically linked together, and over the last years a substantial number of articles in the entrepreneurship literature have studied biotechnology at the regional, firm, and individual level of analysis. This monograph reviews the literature on biotechnology entrepreneurship. First, at the regional level, we focus on innovation networks and biotechnology clusters. Second, at the firm level, we illustrate strategies and business models of biotechnology firms and the determinants of their success. We also elaborate on strategic alliances of biotechnology ventures, and on mergers and acquisitions in the biotechnology industry. Third, at the individual level of analysis, we review the literature on strategic decision making in the biotechnology industry and the role of the management team for biotechnology ventures’ development. We conclude our review by offering future research opportunities within and across levels of analysis for scholars interested in the field of biotechnology entrepreneurship.