先天性高铁血红蛋白症(CongenitalMethe moglobinemia)是较少见的疾病。1884年Francoses首先报告1例,1954年张氏等报告国人1例后,国内虽陆续有报导,但仍未引起临床医生应有的重视,本症易与先天性心脏病(简称先心)相混淆,甚至误行心脏手术。本文报告1例,曾在院外误诊为先心而前来要求手术的病例。王某某、男性、16岁,皖含山县人,住院号28548,出生以来口唇和甲床呈青紫,从无自觉症状,体力活动不受影响,在院外多次被诊断为先心,拟来我院手术治疗。过去身体一向健康,无服用中西药物和接触工 Congenital methemoglobinemia is a rare disease. In 1884 Francoses first reported in 1 case, 1954 Zhang et al reported 1 case of Chinese, although the country has been reported, but it has not yet caused the attention of clinicians, this disease is easily associated with congenital heart disease (referred to as the heart) phase Confuse, or even misconceived heart surgery. This article reports 1 case, who had misdiagnosed as a preoccupation with surgery outside the hospital. Wang Moumou, male, 16 years old, Anhui Hanshan County, hospital number 28548, lips and nail bed since birth was purple, no symptoms, physical activity is not affected, many times in the hospital was diagnosed as a congenital heart, Surgical treatment in our hospital. In the past the body has always been healthy, without taking Chinese and Western medicines and contact workers