Purpose: Extrapulmonary dissemination of Coccidioides species is associated with significant morbidity and mortality. The clinical manifestations vary widely according to the host, the severity of illness, and location of dissemination. The morbidity and mortality can be reduced by early recognition and treatment, which in turn depends on understanding the spectrum and presentation of disease. Methods: We performed a retrospective analysis of 150 cases with extrapulmonary nonmeningeal disease seen from 1996 to 2007 at a referral medical center in an endemic region. Results: Hematogenous dissemination was associated with high mortality and occurred primarily in immunocompromised patients, but only 30% of patients with more limited forms of dissemination were immunocompromised. In keeping with prior studies, there was a preponderance of males (nearly 2:1) and people of African or Asian (especially Pacific Islanders) descent. In contrast, Hispanics and diabetics were not at increased risk. Serology was frequently negative in immunocompromised patients, but the diagnosis could be established by isolation of the organism in culture, or in histologic or cytologic specimens. Conclusions: Although coccidioidomycosis is a great imitator, the diagnosis can usually be made readily if a high level of suspicion is maintained and appropriate diagnostic testing is performed. In most patients, that will include serologic testing in addition to cultures and histology or cytology of appropriate samples.