Vision was 6/60 in the left eye and nystagmus prevented detailed slit lamp biomicroscopy. Under anaesthetic we documented iris hypoplasia and trans-illumination, perinuclear cataract, mild lens subluxation and globular anterior vitreous opacities (Figure 2). Retcam imaging showed that these opacities were in the anterior vitreous which also had liner opacities(Figure 3). Indentation indirect ophthalmoscopy confirmed retinal elevation supero-temporally with OCT confirming a diagnosis of retinoschisis and posterior staphyloma (Figure 4 A,B)). General examination showed an circular defect in the occipital scalp(Figure 5). The clinical feature were consistent with a diagnosis of Knobloch's syndrome. A conservative plan of management was agreed involving regular imaging with the Optomap.