ABSTRACT
We report here the case of a 5 year old female child who presented at Eye department of Abbasi Shaheed Hospital with gradually increasing proptosis of left eye ball since birth. There was no light perception in the eyeball. Examination revealed a cystic swelling in inferolateral part of the orbit causing superomedial displacement of the orbit with restriction of extraocular movements. In addition there was microcornea, miccrophthamos and coloboma of iris extending posteriorly to the optic nerve. Right eye showed microphthalmos, microcornea, nystagmus on attention and a reacting pupil with visual acuity of finger counting at two feet. CT scan showed a large smooth walled hypodense lesion arising from inferolateral aspect of the orbit displacing the eye ball superomedially along with remodeling of adjacent bones.The cyst was successfully removed through subcilliary incision under general anaesthesia. Continuation of the cyst with optic nerve was seen peropeatively. Histopathology revealed a benign cyst with lining of single layer of epithelium and wall composed of aggregates of lymphocytes congested blood vessels and skeletal muscles. Findings were consistent with colobomatous cyst of the orbit