Introduction: Retinoblastoma is the most common intraocular tumor in child. The treatment is well codified combining chemotherapy, surgery, radiotherapy and conservative treatment. It involves both the visual and vital prognosis. The aim of this study was to describe epidemiological aspect of retinoblastoma and evaluate therapeutic aspects and evolution of retinoblastoma in Madagascar. Method: We report in this retrospective study 52 cases found during 5 years, from January 2010 to December 2015. We have collected information about epidemiological and clinical aspect. Delay of diagnosis, treatment done in children and evolution. Data was collected with excel software and analysis was done with Epi info 7.0 software. The quantitative variables were expressed as an average and the qualitative variables as a proportion and percentage. Result: We had collected 52 cases. The incidence was 10.4 cases per year. Mean age was 3 years, with no gender predominance. More than half or 53.8% (n=28) of children presented minor signs: including leucocoria in 25 children and strabismus in 3 children. Exophthalmos was found in 46.5% (n=24) of cases. Exophthalmia and leukocoria were associated in 6 (10%) children. The delay time before diagnosis was 11 months and 2 days. 39 pieces were sent for examination. Result of Anatomo-pathological study was: 51% (n=20) was undifferentiated retinoblastoma, 36% (n=14) differentiated retinoblastoma, 13% diffused retinoblastoma (n=5) Optic nerve was affected in 7.69% of cases (n=3). Before diagnosis, 55.8% (n=29) of our patients received non-specialized medical treatment and 44.2% (n=23) used traditional treatment. Four patients had primitive enucleation without first chemotherapy. Nine children or 18.75% didn’t have surgical treatment. Neoadjuvant chemotherapy was done in 92.3% (n = 48). 39 patients had first chemotherapy before enucleation. Only 58% of children have received adjuvant chemotherapy. The evolution was marked by 27% (n = 14) of remission, 21% (n = 11) of death and 8% (n = 4) of recurrence. Twenty-three patients (44%) were lost to follow-up. Conclusion: Diagnosis of retinoblastoma was made in advanced stage. Number of lost to follow-up were high. Treatment consist in chemotherapy and enucleation. Prognosis was worse in Madagascar. Communication with parents, medical personal must recommended to reduce number of children lost to follow-up.