Introduction: retinoblastoma is a primary malignant intraocular neoplasm that arise from immature retinoblasts with in developing retina. The commonest presenting sign in developing country is proptosis which is the late presenting sign. We report presenting signs of retinoblastoma in Ethiopian children seen at a tertiary level teaching hospitals in Ethiopia.

Methods: prospective case series study wasdone on children who presented with retinoblastoma between May 1, 2005 and September 1, 2006. This study was done as part of requirement for partial fulfilment of certificate of speciality study in ophthalmology during the year 2005 to 2006. SPSS 11 statistical package was used to analyse the data.

Results: among 41 patients seen during the study period, 24 (58.5%) were males and 17(41%) were females. Unilateral retinoblastoma wasfound in 32 (78%) patients and bilateral cases were found in 9(22%). Mean age of onset for right eye was 27.5 months and left eye 33.7 months.The mean ages of presentation at time of diagnosis for right and left eye were 34.4 and 40.2 months, respectively .In bilateral retinoblastoma mean age of presentation was 33.3 months.The commonest presenting sign was proptosis 22(53.7%) followed by leucocorea nine (22%),ocular inflammation four (9.0 %), strabismus three (7.3%), glaucoma one (2.4%), loss of vision one (2.4%)and hyphemaone (2.4%).

Conclusion: the commonest presenting signs of retinoblastoma in our set up were Proptosis followed by leucocorea. This is due to late presentation of patient and late referral by medical professionals. Health education to the public and health professionals will help early detection of retinoblastoma.