This case documents unilateral congenital glaucoma associated with congenital lower lid entropion. A 2-year-old female infant was referred for evaluation and treatment of right-side buphthalmos caused by congenital glaucoma associated with bilateral congenital lower lid entropion that was prominent on the right side and present at birth. Examination disclosed a lower eyelid entropion of the right side that was treated surgically by reinserting the disinserted retractor aponeurosis to anterior inferior tarsal border. After three weeks, the patient was successfully treated with primary combined trabeculotomy-trabeculectomy for congenital glaucoma. The entropion of the left lower lid was asymptomatic and did not require any surgery. Buphthalmos caused by congenital glaucoma may be associated with congenital lower lid entropion and the association may be causal or coincidental.