BACKGROUND Pterygia are characterised by progressive growth of fibrovascular tissue over the nasal limbus towards the corneal center with the loss of Bowman's layer. We report on three patients with bilateral symmetrical affection of the nasal and temporal limbus in one family. PATIENTS A 81-year-old female, her 61-year-old son and her 37-year-old grandson presented with a familiar form of pterygium-like corneal changes. Remarkable was the bilateral symmetrical affection with involvement of the nasal and temporal limbus. Both male patients had multiple excisions (including lamellar keratoplasty) but suffered from persistent recurrences, which led to sustained reduction of the visual acuity. Histological examination revealed typical elastoid degeneration of collagen fibres. CONCLUSION We assume autosomal dominant heredity with varying expression for this "pterygoid corneal dystrophy". It does not seem to differ histologically from normal pterygia, but recurrences appear to be more frequent and more aggressive.