The clinical manifestations of Langerhans cell histiocytosis have been recognized for more than a century. For most of that time, physicians have viewed the disease from different perspectives, interpreting portions of its clinical spectrum as if they were distinct and unrelated entities. More recently, Langerhans cell histiocytosis has been unified into a single concept, though the disease continues to defy traditional classification. By most accounts, Langerhans cell histiocytosis appears to be a morphologically benign proliferation of inflammatory cells that escapes regulatory control mechanisms. Studies from patients with all stages of the disease, however, document clonal proliferation of immune processing cells (i.e., Langerhans cells), suggesting a malignant disease process. The most common ophthalmic manifestation of Langerhans cell histiocytosis is a solitary lesion of orbital bone, which typically responds to minimally invasive therapy. The best management of solitary orbital Langerhans cell histiocytosis is debatable and has been complicated by its recent designation as a risk factor for central nervous system disease. This article summarizes recent developments in understanding the biology of Langerhans cell histiocytosis, reviews its ophthalmic manifestations, prognosis, and the controversy surrounding treatment of isolated orbital disease.