The clinical, ultrasonographic, CAT scan and pathologic findings are described in a case of a rare unilateral malformation involving the optic disc of the type described as the 'morning glory syndrome'. The malformation was associated with a peripapillary scleral defect, absence of the lamina cribrosa and a recess formed by an axial retrodisplacement of the optic nerve. There was an associated secondary exudative retinal detachment with retinal atrophy and gliosis; the retinal pigment epithelium showed peripapillary proliferation. Additional features included: (a) an apparent schisis in the retina within the retrobulbar recess, (b) an absence of the perineural meninges which were replaced by fibroadipose tissue and bundles of smooth muscle, (c) peripheral optic nerve atrophy and (d) a vascular plexus surrounding the central retinal artery.