Coccidioidomycosis is a fungal infection endemic to the southwestern United States. Typically a respiratory illness, coccidioidomycosis can rarely present as extrapulmonary infection. Skeletal coccidioidomycosis occurs in 20% to 50% of disseminated infections. Skeletal coccidioidomycosis is a chronic and progressive infection that eventually results in bone destruction and loss of function and often involves adjacent structures, such as joints, muscles, and tendons and other soft tissues. Sinus tract formation may occur. This infection may be multifocal. Although radiographs, white blood cell count scans, and other imaging methods identify and define relevant abnormalities, histopathologic examination with culture of the involved bone is the only means to confirm the diagnosis. Serologic testing is adjunctive, and complement fixation titers can be evaluated serially to assess response to treatment. A number of studies addressing the efficacy of various antifungal agents have been performed, and the results of these studies as they pertain to skeletal coccidioidomycosis are summarized herein. Among the various studies, response rates ranged from 23% to 100%, but relapse was common. A combination of medical therapy - often, itraconazole or fluconazole - and surgical d'ebridement is often needed to control skeletal coccidioidomycosis. Early diagnosis and treatment are critical to avoid long-term problems with chronically infected bones and joints. Anatomical issues, diagnostic studies, and data related to treatment of this form of extrapulmonary coccidioidomycosis are reviewed in this article.