BACKGROUND Conjunctival myxomas are rare, benign, connective tissue tumours that classically present as slow-growing, painless, well-circumscribed masses (Arch Ophthalmol 124:735-8, 2006; Case Rep Ophthalmol 3:145-50, 2012). There have been 29 cases reported in the literature (Arch Ophthalmol 124:735-8, 2006; Malays J Med Sci 20(1):92-4, 2013; Case Rep Ophthalmol 3:145-50, 2012; Middle East Afr J Ophthalmol 19(3):353-3, 2012). We present a case with atypical features, and emphasize the importance of excisional biopsies for diagnosing indeterminate conjunctival lesions. CASE PRESENTATION A 32 year old Korean woman presented with a 5 mm × 7 mm × 3 mm pedunculated firm cystic lesion on the inferior palpebral conjunctiva of her right lower eyelid. The lesion had rapidly enlarged over the course of a week. She gave a history of uncomplicated bilateral epiblepharon correction performed in Korea three months prior. There were no systemic features, or family history of genetic conditions. The lesion was excised under local anaesthesia and reported to be a conjunctival myxoma. The clinical and histopathological features of this lesion were consistent with previous reports on conjunctival myxoma (Arch Ophthalmol 124:735-8, 2006; Arch Ophthalmol 101:1416-20, 1983; Case Rep Ophthalmol 3:145-50, 2012; Am J Ophthalmol 102(1):80-84, 1986). The unusual features of this case were, the rapid growth of the lesion - with the previously documented mean time before presentation being 34 months (range 3 months - 24 years) (Arch Ophthalmol 124:735-8, 2006; Case Rep Ophthalmol 3:145-50, 2012); the location of the lesion in the inferior palpebral conjunctiva - 93 % of previously reported cases had occurred in the bulbar conjunctiva (Arch Ophthalmol 124:735-8, 2006; Case Rep Ophthalmol 3:145-50, 2012); and its occurrence in association with recent eyelid surgery - which has never been reported. CONCLUSION This case of conjunctival myxoma adds to the small number of documented cases, by demonstrating an atypical presentation. Conjunctival myxomas can occur in association with the Carney Complex, which is an autosomal dominant syndrome associated with benign tumours, spotty mucocutaneous pigmentation, and endocrine overactivity (Ophthalmic Surg Lasers Imaging 39(6):514-6, 2008). Ophthalmic manifestations of the Carney Complex have been found to precede vascular embolic events secondary to cardiac myxoma, thus early diagnosis of conjunctival myxoma can prevent potentially devastating consequences (Ophthalmic Surg Lasers Imaging 39(6):514-6, 2008). The different presentations of this rare tumour emphasise the importance of excisional biopsies in diagnosing indeterminate conjunctival lesions; and its association with cardiac myxoma, highlights the need for cardiac investigations in all patients who present with conjunctival myxoma (J Ophthalmol (1);1-5, 2014; Ophthalmic Surg Lasers Imaging 39(6):514-6, 2008).