The purpose of this study is to review the literature on microsporidiosis in various high-risk groups among the Malaysian population, i.e., HIV/AIDS, cancer, hospitalised patients and Orang Asli, and to update information with regards to microsporidia prevalence, diagnosis and association of the disease with gastrointestinal symptoms in Malaysia. Hospitalised patients showed the highest prevalence (28.3%) of microsporidiosis compared to other risk groups. This review did not find any direct correlations between gastrointestinal symptoms and microsporidiosis. Since microsporidiosis is an emerging threat to the high-risk groups, greater awareness should be instilled among clinicians to consider microsporidiosis in their differential diagnosis if no other causes can be defined.