Background: In developed countries, retinoblastoma is curable in more than 95% of cases, whereas in low-income countries, mortality remains high, especially when the diagnosis is made late or the treatment is discontinued. The aim of this work was to determine the factors associated with adherence to the treatment of retinoblastoma in the Ivory Coast and the Democratic Republic of Congo (DRC). Procedure: A retrospective cohort study was carried out. Data were collected from patient folders and follow-up records of parents. Results: A total of 175 children with retinoblastoma were registered from January 2013 to December 2015. Seventy-six children (43%) were 5 years old and above. Care costs were covered by families in 86.9% of cases. Chemotherapy refusal was recorded in 39 cases (22.3%), and enucleation refusal was recorded in 79 cases (45.1%). After 36 months of follow-up, we recorded 16.6% deaths, 27.4% treatment dropouts, and 18.3% loss to follow-up after treatment. The commonest cause for enucleation refusal was fear of infirmity, while chemotherapy refusal and absconding treatment were due to financial constraints. Conclusion: Poor adherence to retinoblastoma management was due to financial constraints, and a lack of knowledge of the disease and its treatment. Family psychosocial support is needed to improve this condition.