Posterior lenticonus (PLC) is a type of congenital lens disease in the morphology of spherical or conical bulging of the posterior capsule of the lens.It occurs predominantly in unilateral and sporadic, and the bilateral case is often inherited in type of autosomal dominant or X-chromosome linked inheritance.The mutations of EPHA2 are the major known causes of PLC until now.Although researchers have proposed hypotheses that the formation of PLC are related to weak posterior capsule, excessive growth of lens epithelial cells and traction of pigment tissue based on the clinical manifestations of PLC, the pathogenesis of PLC is not elucidated.The animal models associated with PLC can be roughly divided into posterior capsule protrusion and posterior capsule rupture combined with cataract.The clinical manifestations of PLC are various with main symptoms including leukocoria, strabismus, photophobia, and decreased vision.Preoperative examinations by slit-lamp microscopy, ultrasound biomicroscopy, Pentacam can detect abnormal morphological changes of the posterior capsule of lens.Intraoperative characteristic manifestations such as fish-tail and jelly-fish sign can be used to diagnose PLC.When PLC is combined with lens opacity, cataract extraction combined with posterior continuous curvilinear capsulorrhexis or posterior capsule cutting with vitrectomy is the main surgical treatment.Intraocular lens implantation or glass wear is used to correct the aphakic eyes.Postoperative amblyopia training is crucial for improving visual function.