BACKGROUND፡ Retinoblastoma (RB) is one of the most curable childhood cancers if early detected and treated. Late presentation complicates the management of RB results in dismal treatment outcome. Objective: The aim of this study is to report the clinical presentation pattern of retinoblastoma patients seen at Jimma University Medical center (JUMC).METHODS: The study was a retrospective review of retinoblastoma cases managed at JUMC between August 2016 and July 2020.RESULTS: Among pediatric oncology cases registered retinoblastoma, accounting 8.5 % (36/423) of all childhood cancer patients in the study period, 29 (90.6%) of them had unilateral retinoblastoma and 3(9.4%) of them had bilateral retinoblastoma. The average age at presentation for bilateral and unilateral retinoblastoma patients was 17 (range 3-30) months and 37.5 months (range 8-84) respectively. The first symptom observed by the family was leukocoria in 21 (65.6%) of the patients but 24(75%) of the patients presented with advanced stage (proptosis and fungating orbital mass) of the disease. The longest and the shortest lag time of presentation from the first symptom was 17 months and 2 weeks respectively with the mean lag time of 1.4 months in bilateral and 6 months in unilateral cases. Clinically, the majority of the eyes 24/35(68.6%) were extraocular tumors involving orbital tissues at presentation.CONCLUSION: Most of retinoblastoma patients presented at advanced stage of the disease and presented very late after the family observed the disease. Early detection strategies must be designed by the government and responsible stakeholders in mitigating the effects of late presentation.KEYWORDS: Retinoblastoma, leukocoria, orbital disease