Issues of gender ambiguity have been discussed over time from both Islamic and medical perspectives. In Islam, these issues are typically considered in the context of khunūthah (literally translated as hermaphroditism). While biomedical studies have appeared to provide a large amount of information on abnormal human biological development, i.e. Disorders of Sex Development (DSDs). However, the connection between these two fields has been given little attention. This research aims to determine the Islamic underpinnings through the fatwa around the globe. Thus, institutional fatwa organisations among Sunni schools of thought at the international, regional and national levels are observed. The fatwas regarding the management of individuals with gender ambiguity, not specifically on DSDs, are chosen and presented accordingly. Based on the findings, the sporadic fatwas from different parts of the world delineate the issue of sex ambiguity and seem to be able to provide general guidelines for management of Muslim patients with DSDs. Three common aspects have been discussed including the methodology of gender assignment, the decision-making process and the surgical and hormonal treatments.