Coccidioidomycosis is a fungal infection endemic to the Southwestern United States that has a clinical presentation resembling community-acquired pneumonia. Disease occurs after inhalation of airborne arthroconidia. Dissemination to a variety of organ systems via hematogenous spread from a primary pulmonary focus may then occur. Coccidioidomycosis rarely involves the abdominal cavity. The authors review the spectrum of abdominal and pelvic presentations of coccidioidomycosis and report 6 unusual cases, including acute appendicitis, hepatitis and adnexal masses. Pathologists played a critical role in the diagnosis of these presentations by recognizing spherules in tissue. In only 2 of the cases were Coccidioides species cultured.