Non-Langerhans cell histiocytosis(NLCH) includes various disorders, and histologically they are characterized by dermal collections of cells that are derived from macrophages as well as some cells with dendritic differentiation. Progressive nodular histiocytosis(PNH) is the rarest form of the NLCH. And there are only a few reports of PNH in the English literature, but no case report in the Korean dermatologic literature. A 18-year-old boy had about 2 or 3-year history of firm nodules on the both elbows and knees. The nodules had steadily grown in size and number. On physical examination, there were numerous red to purple nodules on his both elbows and knees. The patient had neither medical problems nor family history, and there was no abnormal finding in the laboratory and imaging studies. On the histological examination, the dermis contained abundant histiocytes with polygonal nuclei and foamy cytoplasms admixed with spindle cells. Prussian blue stain revealed mild interstitial iron staining in the stroma and Oil-red O staining showed a prominent cytoplasmic staining diffusely in the foamy histiocyte. Immunohistology was positive for CD68, while it was negative for SMA. We, herein, report a first case of PNH of 18-year-old boy in Korea.