Gardners Syndrome, a common variant of familial adenomatosis polyposis affects one in approximately 8000 births. This syndrome is identified due to a distinctive triad of familial intestinal polyposis, surface tumours of hard tissue particularly osteomas in the skull and surface tumours of the soft tissue. The intestinal polyps have a 100% risk of undergoing malignant transformation. Hence, early detection of the disease is critical. This article discusses the surgical management of an unesthetic orbital osteoma in a particularly undiagnosed case of Gardners Syndrome.

Keywords: Gardners syndrome: Autosomal dominant; Polyposis; Osteomas; Soft tissue tumours.