A case of unilateral microphthalmos with a sizeable retrobulbar cyst, totally dysplastic retina, and gross nerve head coloboma is reported in an otherwise normal pigmented rabbit. Despite the gross abnormality of the posterior part of the globe, an iris sectoral coloboma, and abnormal lentoid body, the cornea was complete but had moderate edema and a reduced diameter. Scanning electron microscopy and morphometric analysis of the corneal endothelium showed a fully tessellated mosaic of cells that displayed only modest pleomorphism and polymegethism.