Brittle cornea syndrome (BCS) is a rare connective tissue disease, with autosomal recessive inheritance, caused by mutation of ZNF469 and PRDM5 genes. Its main characteristic is severe corneal thinning and weakness, with a tendency for corneal rupture, that occurs spontaneously or after minimal ocular trauma. We present the case of a 4-year-old girl, with corneal rupture in both eyes after minor traumas, and with hypoacusis and joint hypermobility as extraocular manifestations, emphasizing the difficulties of treatment, and discussing the correct denomination of the syndrome as BCS, avoiding the frequent confusion with Ehlers Danlos type VI.