Endemic mycoses are caused by dimorphic fungi and eventually molds, as the case of implantation mycoses. In general, these diseases are acquired through trauma or inhalation of fungal elements in the environment, and less frequently by zoonotic acquisition or transmitted during organ transplantation. The target population for endemic mycoses is usually represented by normal hosts with low-income and intensive outdoor activities. Awareness of these diseases remains limited, even in regions with high prevalence, resulting in delayed diagnosis, and affecting the quality of life and outcomes of patients who suffer from these entities. In this review, we summarized relevant information about epidemiological, clinical, diagnostic, and treatment aspects of the most common endemic mycoses, including blastomycosis, coccidioidomycosis, histoplasmosis, paracoccidioidomycoses, talaromycosis, and implantation mycoses. The main goal of this review is to provide key concepts in terms of when to suspect, how early diagnose, and properly treat patients with these mycoses.