Goldenhar syndrome is well known for its classical triad of epibulbar dermoids or lipodermoids, auricular appendages and pretragal fistulas. Here we report a case of Goldenhar syndrome with an unusual association of a fibroepithelial polyp attached to a limbal dermoid. A case of Goldenhar syndrome in a 5-month-old male infant presented with the features of a fibroepithelial polyp attached to a limbal dermoid, right-sided polydactylia with hypoplastic thumb, and accessory preauricular appendages on the left side. The association of a fibroepithelial polyp attached to a limbal dermoid with Goldenhar syndrome is a rare report in the literature. In these cases, regular follow-up with an ophthalmologist is important to monitor the visual development of the patient. Ideally, a multidisciplinary approach is required to manage the other associated anomalies.