本文报告4岁男孩神经纤维瘤的临床病理改变。其左眼眶神经鞘瘤恶变后部分向横纹肌肉瘤分化,因而在临床上及病理上均误诊为眼眶横纹肌肉瘤。本文讨论了这一罕见肿瘤的临床诊断、鉴别诊断及其治疗。关键词:神经纤维瘤病恶性神经鞘瘤横纹肌肉瘤患儿男4岁左眼球突出3年,最近两个月加重,于1990年2月入院。1岁时,左眼上睑肥厚,眼球突出,左上睑下垂,色深,血管扩张、纡曲,眼球向前、下方移位,向上向外运动受限。外上眶缘扪及 This article reports the clinical pathological changes of 4-year-old boy neurofibromas. The left iliac nerve sheath tumor differentiated into rhabdomyosarcoma after malignant transformation, and thus was clinically and pathologically misdiagnosed as orbital rhabdomyosarcoma. This article discusses the clinical diagnosis, differential diagnosis, and treatment of this rare tumor. Keywords: Neurofibromatosis Malignant schwannomas Rhabdomyosarcoma in children Male 4-year-old The left eyeball protruded for 3 years and was aggravated in the last two months. He was admitted to hospital in February 1990. At the age of one year, the upper eyelid was hypertrophic, the eyeball was prominent, the left upper eyelid was sagging, the color was dark, the blood vessel was dilated and twisted, the eyeball was displaced forward and downward, and the upward and outward movement was limited. On the outside