Retinoblastoma is the most common primary intraocular malignancy in children, with approximately 100% survival rates in higher-income countries. The outcomes of retinoblastoma depend on factors such as disease presentation, treatment access, histopathological high-risk factors, and national income level. This retrospective study analyzed 12-year data (2012 – 2023), including presentation, histopathological features, histopathological high-risk factors, and survival outcomes, of 78 retinoblastoma patients who had undergone primary enucleation at a tertiary eye center in Bangladesh. The overall median age was 33 months, with a slightly older median age among females (35 months) and a mild male predominance. The mean symptom duration was 5.68 ± 3.56 months, with earlier presentation in male patients. The most common presenting sign was leukocoria (70.5%), followed by red eye (20.5%). Combined endophytic and exophytic tumor growth (36%) and poor differentiation (44.9%) were prevalent. High-risk factors were present in 84.6% of patients, with massive choroidal invasion (41%) and retrolaminar optic nerve invasion (38.5%) being the most frequent. Approximately 57.7% of patients solely underwent enucleation, and 27% of children died, yielding an overall survival rate of 69.23%. The findings of this study suggest that multiple high-risk factors are often present in advanced tumors, posing challenges in management and increasing the risk for metastasis. Efforts to improve outcomes should focus on raising awareness, promoting early presentation, enhancing histopathological reporting, and developing specialized human resources.