Abstract : Primary congenital glaucoma (PCG) is a common disorder in Saudi Arabia. Stromal Oedema owing to leakage of aqueous humour through a tear in Descemet's membrane (DM) is charachtaristic of acute corneal hydrops, and has been reported in cases of PCG. The question do these eyes react differently after this. Wether the inheritance of acute DM break in acute corneal hydrops or the eyes reacts differenetly from congenital glaucoma seems uncertain. In this report, we describe a case of a 13-day-old child who presented with a diagnosis of possible unilateral corneal ectasia. He had keratoglobus and corneal hydrops in the right eye, no significant prenatal/postnatal history, and positive consanguinity. His right eye had normal intraocular pressure, enlarged horizontal corneal diameter, and elongated axial length with no view of the fundus. Examination of his left eye showed possible glaucoma (low hyperopia), despite normal intraocular pressure. Genetic testing confirmed a homozygous CYP1B1 mutation (G61E). This case sheds light on the varied presentations of patients affected by the CYP1B1 mutation.