Cyclosporiasis is an emerging disease that is ubiquitous in humans and other animals worldwide. Cyclospora cayetanensis, which is distributed in the tropical and subtropical regions, and endemic in several developing countries, is the only species of Cyclospora resulting in cyclosporiasis in humans. The main objective of this review was to summarize the prevalence of Cyclospora in humans, animals, and environmental samples in Nepal. The overall prevalence of Cyclospora in humans has been reported to be 10.3% (3,785/36,570), including 3.6% (60/1,640) among HIV immunocompromised cases, 2.1% (210/9,733) among children, and 13.9% (3,515/25,197) in humans of all age categories, while the prevalence was calculated to be 2.9% (29/983) and 10.5% (9/85) in domestic and wild animals, respectively. The molecular characterization data on Cyclospora as related to Nepal is limited to a very few positive isolates from humans, dogs, chickens, and monkeys. Exposure to sewage water and contaminated fresh food items, and the presence of domestic animals appear to have been associated to an outbreak of Cyclospora in humans. Considering Cyclospora spp. as the etiologic agent for diarrhoea, a comprehensive, wider scale molecular epidemiologic study in humans, animals, and environmental samples is warranted to elucidate the exact distribution loci and probable outbreak of cyclosporiasis in Nepal.