Posterior microphthalmos is a congenital anomaly of the posterior segment of the eye, caused by an abnormally thickened sclera. The thickened sclera does not impede the growth of the neuro-retina but seems to influence the development of the choroid and of the retinal pigment epithelium. This may explain the common occurrence of a papillomacular fold in such cases. As such eyes are at risk of developing uveal effusion or angle-closure glaucoma, it is important to consider the diagnosis of posterior microphthalmos in high hypermetropic eyes.