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The ocular features of Goldenhar syndrome (GS) are typically external, like surface dermoids and lid coloboma. Retinal detachment (RD) is rare and has not been reported in absence of other concomitant predisposing congenital ocular disorders. We present a unique case of bilateral rhegmatogenous retinal detachment (RRD) with GS. To the best of our knowledge, this association of GS with RRD is novel and has not been reported earlier in ophthalmic and systemic literature on RRD.