作者报道了5例先天性鼻梨状孔狭窄(CNPAS)伴上颌单一门齿(SCMI)的CT及MRI表现。男3例,女2例,年龄11天～8岁。主诉呼吸困难4例,身材矮小1例。CT检查5例,采用2～4mm层厚连续常规横断面扫描,扫描层面与硬腭平行,部分病例作了冠状位扫描。MRI检查2例,采用自旋脉冲序列,均作了横断面、冠状位和矢状位的T_1WI,T_2WI和PDWI。CT显示CNPAS 5例,未长出 The authors reported the CT and MRI findings of 5 cases of congenital nasal tap hole stenosis (CNPAS) with maxillary single incisor (SCMI). 3 males and 2 females, aged 11 days to 8 years old. Chief complaint of dyspnea in 4 cases, short stature in 1 case. CT examination in 5 cases, using 2 ~ 4mm thickness continuous conventional cross-sectional scan, the scan level parallel with the palate, some cases were coronal scan. Twenty-one patients were examined by MRI, T_1WI, T_2WI and PDWI were used as cross-sectional, coronal and sagittal images. CT showed CNPAS 5 cases, did not grow