Neurofibromas are developmental disorders of nerve sheaths and usually not well circumscribed or encapsulated, although they present as tumor-like formations. The tumor mingles with the surrounding dermal and other tissue, or it involves diffusely for variable distances. Neurofibromatosis was first described by von Recklinghausen in 1882 and is characterized by cafe-au-lait spots in the skin, wide disseminated neurofibroma of central and peripheral nervous system with variable size, its hereditary and chronic progressive nature. This disease is occasionally associated with anomalies of skeletal system, gastrointestinal system, genitourinary system and endocrine system. Rarely, when such tumors become malignant, they become fibrosarcoma, liposarcoma, myosarcoma, squamous cell carcinoma, adenocarcinoma of gastrointestinal system, etc.
Two cases of giant neurofibroma in head including one case of von Recklinghausen’s disease were encountered, which cause severe deformity of facial configuration.
Surgical excision was performed and then two patients live with relatively social acceptable appearance.
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