A coccididomycosis is a kind of systemic fungal infection, respiratory in origin, that spreads hematogeneously to skin, subcutaneous tissue, bone, CNS, and other organs. The author here present a case of systemic coccidioidomycosis with skin involvement which has been found in Dec. 1974, for the first time in Korea. The coccidioidomycosis is proved through skin biopsy and mycotic culture from skin lesion. The patient was 57 year old Korean male whose occupation was a cattle breeder. The skin lesion on left extremity showed several skin colored or light brownish papules and surrounding circular erythematous scaly patches with slightly elevated borders. The patient also found to have diabetes mellitus and resultant neuropathy. Histologic picture of skin biopsy showed granuloamatous lesion in the upper dermis and numerous spherules both inside and out side of giant cells. Mycologic culture from grind skin specimen on Sabouraud’s agar showed snow white colony and typical arthrospres.