Background: Retinoblastoma (Rb) is rare malignant tumor that occurs mainly in children under age 5. Has a poor survival prognosis in Africa (as opposed to Rb in developed countries) leading to loss of life within 1 to 2 years if untreated. It is the most common primary intraocular malignancy in childhood, accounting for 3%of childhood malignancies. In developed countries, the rate of survival for a child with retinoblastoma is 95%, but in developing countries the reality of retinoblastoma is still a challenge due to late presentation, late pathological result,ignorance,less diagnostic options optimal treatment and poor treatment adherence. As a result the survival rate in developing countries is almost 0%. In Tanzania, only a few studies (audits) have been done on prevalence and management of Retinoblastoma. Currently the National Eye Care Project coordinator (MoH) together with teams from Muhimbili National Hospital, KCMC and Bugando Hosp., are preparing a National Retinoblastoma Guide, this being an important event among the measures taken for improving the prognosis, the survival rate in particular. This study will be aiming at determining the prevalence and management ofRb in under five children attending the Ophthalmology Department at KCMC in Moshi. Objectives: The specific objectives for the study were to determine the prevalence, its social demographic characteristic,clinical presentation as well as to assess management and treatment adherence of retinoblastoma among under five children attended Ophthalmology department at KCMC Methodology: This was be a retrospective hospital based study. The study will focus on the prevalence and management of Rb in under five children attending Ophthalmology Department at KCMC Moshi. Key information using questionnaires will be obtained from the files in Eye Medical Records. Result Total of 47 children‟s where confirmed to be retinoblastoma on ophthalmology department at KCMC, females were 22 of 47 and male were25 0f 47 approximately to be46.8% and 53.2% respectively. The mean age of 25.23 (SD +_13.2) months. Most of children was between 13-24 months approximately 36.2 %. Children‟s who has bilateral presentations where 5 and Unilateral presentations where 42 children‟s. In our study one participant can present with more than one sign, 39 of 47children‟s was presented with Leukocoria (83%) which is the commonest presentation, other 28 of 47 children’s with proptosis (59.9%) and 10 of 47 with strabismus 21.3%.Enucleation was treatment of choice in most of participant 32 of 47 (68.1%). CONCLUTION Retinoblastoma is a rare tumor in under five children but still a major problem in our community. Management of it still needs multidisciplinary team work which starts from the community, parents or care takers and health professionals by creating awareness of presenting signs, importance of early diagnosis, and early referral to tertiary hospital, in order to reach first goal in treatment which is blindness prevention.