Abstract
Langerhans cell histiocytosis is a progressive proliferative disorder of mononuclear macrophage system. The clinical presentation of this disease depends on the extent of dissemination, and the clinical course of the disease is usually variable. In this report an eight-year Sudanese female child
referred to the Oncology out Patient with multiple swellings in her head and thigh, generalized lymphadenopathy and hepatomegaly. Neurologically she had focal abnormal rapid and regular right upper limb movement, persisting even during sleep. Histopathology examination of the biopsy of the thigh swelling showed fibro-fatty tissue with centrally necrotic collection of cells mainly esinophils, plasma cells and histiocytic cells which was consistent with LCH. Multiple lytic lesions were noticed on radiological studies. She was treated . with a regimen of Vinblastine, Etopside and Dexamethasone.

Key words: Langerhans cells, Histiocytosis x, Eosinophilic granuloma, Lettere-Siwe disease, Hand Schuller- Christian disease, Hashimoto-Pritzker syndrome self-healing histiocytosis, pure cutaneous histiocytosis, Langerhans cell granulomatosis, Type II histiocytosis, and the generic term nonlipid reticuloendotheliosis.