Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic Nerve Head Drusen (ONHD) are white calcareous deposits that are asymptomatic. We report a rare association of both in healthy patient with no any systemic syndrome.

A16-year-old man was referred to us from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly

Ultrasonography showed echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and superior field depression in the right eye corresponding to inferior coloboma in funduscopy

Combination of bilateral ONHD and unilateral chorioretinal coloboma in healthy patient with no any systemic syndrome has not been published in literature. We reported this rare association and should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by clinical geneticists