报告1例罕见的先天性鼻侧喙伴先天性半缺鼻畸形,结合文献复习,认为:本病主要表现为一侧鼻侧有管状物,并流出少涕样分泌物。同侧外鼻发育很小或缺如,鼻孔、鼻泪管缺如或闭锁。临床应与脑膜-脑膨出等鉴别。泪道冲洗、泪道同位素显象、X线拍片、侧喙碘油造影及盲管分泌物实验室检查等对鉴别诊断十分重要。冶疗主要为手术整复。并对其发病学等进行了讨论。 Report of a rare case of congenital nasal beak with semi-nasal beak deformity, combined with the literature review, that: The disease is mainly manifested as one side of the nasal tube, and the flow of small tears-like discharge. Ipsilateral nasal development is small or absent, nasal, nasolacrimal duct absence or atresia. Clinical and meningeal encephalocele and other identification. Lacrimal duct lacrimation, lacrimal duct isotope imaging, X-ray film, side beaked lipiodol angiography and blind tube secretions laboratory tests on the differential diagnosis is very important. Mainly for the treatment of plastic surgery. And discussed its pathogenesis and so on.