SUMMARYThe case of twin-sisters, 24 years old, showing congenital squint, that was corrected by surgery, hypermetropia and astigmatism is presented. The ophthalmoscopic examination revealed in one eye of both sisters, and specular-like, tortuosity of the retinal vessels, which in a subject is accompanied by twisted arteries in the vitreous body; in the other twin-sister an epipapillary membrane was observed, which is present in analogous form also in a single born brother. Furthermore, dystrophic lesions of the retina were present specular-like in both sisters. It is the first time that such a malformative syndrome is observed in concordant MZ twins.