In a 9-year-old girl, the ophthalmological examination showed blurred vision in the left eye lasting more than 6 months. A port wine birthmark was visible on the right side of the face. Visual acuity was normal in the right eye (20/20) but markedly reduced in the left eye for counting fingers, improving to 20/1000 with correction. Funduscopy revealed a diffuse choroidal hemangioma in the right eye and an enlarged optic disc with a central funnel-shaped hollow, radiation vascular anomalies, and retinal detachment in the left eye. They diagnosed Sturge-Weber syndrome (SWS) affecting the right eye and contralateral morning glory syndrome (MGS) with retinal detachment in the left eye. This is the first reported case of coexistence of SWS and MGS in one patient, confirmed by imaging methods. A thorough examination of the contralateral eye and a neurological examination are essential in patients with a port wine birthmark or unilateral optic disc anomalies.