Histiocytosis sometimes involves the joints, and is one of the important differential diagnoses of osteoarthropathy. A 31-year-old man presented with recurrent fever and bilateral knee arthritis two years prior to admission. He also showed the hypopituitary mass lesion and partial hypopituitarism. X-ray studies showed both osteosclerotic and osteolytic lesions near the large joints. Histological findings of bone biopsy revealed foamy macrophage infiltration, which were CD68+CD1a-S100-, and Erdheim-Chester disease was diagnosed. In addition, CD68+CD1a+ Langerhans' cells also aggregated in the same lesions, and we thought this case was a rare variant of Erdheim-Chester disease with overlapping histiocytic invasion.