Coccidioidomycosis, an infection caused by Coccidioides spp., is traditionally recognized as an endemic fungal disease mainly restricted in part of North America. However, imported cases in non-endemic areas are increasingly reported, indicating a paradigm shift on the epidemiology of coccidioidomycosis. The clinical manifestations of coccidioidomycosis vary significantly, ranging from mild self-limited upper respiratory infection to severe disseminated ones, leading a challenge for the diagnosis of the disease. Although China is not an endemic area of coccidioidomycosis, it is important for domestic healthcare providers to stay alert of the disease. This review aims to summarize the current advances in the epidemiology and clinical aspects of coccidioidomycosis.