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Axenfeld–Rieger syndrome (ARS) is a rare disease characterized by anterior segment anomalies with or without glaucoma. A 28-year-old antenatal female diagnosed with ARS presented with uncontrolled intraocular pressures (IOPs) and advanced glaucoma despite maximal medical therapy and progressive vision loss during her third trimester. The refractory and progressive nature of the disease, with useful vision in one eye, necessitated immediate surgical intervention, following which there was successful control of IOP, lasting till the final follow-up at 18 months. We discuss the role of glaucoma surgery, for an ARS patient with refractory glaucoma during the third trimester with a comprehensive review of literature.