Cardiopulmonary bypass (CPB) in a patient with glaucoma is a challenge. The glaucomatous eye is at risk during CPB. We report a case of ostium secundum atrial septal defect that was not amenable to device closure. The unique feature in the patient was the presence of congenital glaucoma. She was blind in the left eye, and the visual acuity in the other eye was decreased because of glaucoma. She underwent direct closure of the atrial septal defect under CPB and fibrillatory arrest, with intraoperative monitoring of intraocular pressure. There was no change in visual acuity after 1 year of follow-up.