Myxoma is a benign neoplasm derived from primitive mesenchyme usually occurring in heart but can arise rarely in orbit, eyelid and conjunctiva. A 61-year-old female presented with a history of right eye lower lid lesion of two year duration. The patient had undergone excision of the lesion at a SRTR hospital. Examination showed a soft non tender nodular skin coloured lesion of 2 x2 cm dimension on the lateral aspect of the right lower eyelid. Visual acuity was 6/18, N8. Rest of the anterior segment was within normal limits. Wide excision of the lesion along with lid margin and lid reconstruction was done. Histopathology of the excised lesion showed features suggestive of myxoma with a prominent vasculature. Detailed cardiac and dermatological evaluation did not show other components of Carney’s complex. Isolated eyelid myxoma is an extremely rare condition with a tendency towards local recurrence following incomplete excision