Coccidioidomycosis is an endemic systemic mycosis of the western hemisphere that has acquired mayor relevance after a raise in cases in the recent years. Two species, Coccidioides immitis and C. posadasii are recognized as the causative agents of this disease that, in principle, primarily affects the lungs. Extra pulmonary cutaneous forms have been more frequently reported and its manifestations present a vast clinical spectrum that resembles subcutaneous mycosis, cutaneous tuberculosis or even skin cancer. The interaction of the host and its immune response against the fungus and its pathogenic mechanism play a major role in the evolution, clinical and histopathological aspects and finally in the resolution of the disease.