Infections with geographically constrained dimorphic fungi cause the endemic mycoses, which include blastomycosis, coccidioidomycosis, emmonsiosis, histoplasmosis, paracoccidioidomycosis, sporotrichosis, and penicilliosis. In the last 5 years, our understanding of the epidemiology, diagnostics, and to a lesser extent management of these diseases has advanced. Specifically, the application of molecular techniques for genotyping fungal pathogens has resulted in the recognition of cryptic species within several genera, including Blastomyces, and Paracoccidioides; the reclassification of Penicillium marneffei, the agent of penicilliosis, to the genus Talaromyces; and the global emergence of dimorphic fungi of the genus Emmonsia, cause disease in immunocompromised persons. New and refined diagnostic tests are available based on the detection of circulating antigens and antibodies, mass spectrometry, and targeted gene amplification. In contrast, the development of new therapeutic options remains stalled, although isavuconazole may hold promise. Finally, advances have been made in the prospect of viable vaccines for preventing animal and human disease.