Langerhans cell histiocytosis of bone causes bone destruction and frequent bone reactions that mimic benign and malignant neoplasms as well as osteomyelitis. The bony lesion of Langerhans cell histiocytosis consists of histiocytes with variable numbers of eosinophils, lymphocytes, and neutrophils. We present a rare case of Langerhans cell histiocytosis of bone, including imaging studies and pathology. In addition, we discuss the classification and pathology of Langerhans cell histiocytosis.