A 14-month-old male infant was brought by parents for redness of the right eye of 18 days duration. Exam and B-scan ultrasonography revealed total hyphema, dense vitreous haemorrhage and lens subluxation in the right eye while CT disclosed right small epidural haematoma. The left eye had neither retinal haemorrhage nor disc oedema. There was no sign of shaken baby syndrome. Fibrinogen level in the blood was very low. The parents are first-degree cousins with two family members having hypofibrinogenaemia. Vitreous haemorrhage recurred after surgical intervention resulting in phthisis and loss of vision. Hypofibrinogenaemia needs to be included in the differential diagnosis of ocular haemorrhage and vision loss.