Background: Retinoblastoma remains the most common intraocular malignancy of childhood, and despite being a potentially curable disease, delayed presentation and advanced stage at diagnosis continue to contribute to poor outcomes in low- and middle-income settings. The purpose of this study was to evaluate the clinical presentation patterns and identify prognostic indicators in children diagnosed with retinoblastoma. Methods: This hospital-based descriptive study at the Department of Ophthalmology, National Institute of Ophthalmology and Hospital, Dhaka (April 2011–March 2013) included 46 children with retinoblastoma. Demographic and clinical data, presenting features, lag time, and laterality were recorded. Tumors were staged per eye using IIRC (A–E) and categorized as early (A–C) or advanced (D–E). Management included enucleation or eye-preserving approaches, and data were analyzed using descriptive and comparative statistics to identify factors associated with advanced disease and enucleation. Results: Among 46 children with retinoblastoma, most were 1–3 years old (56.5%), male (56.5%), and had unilateral disease (71.7%). Leukocoria was the most common presentation (69.6%), followed by leukocoria with proptosis (15.2%). Over half presented within 3 months of symptoms, while 21.7% had >6 months’ delay. Advanced tumors (IIRC D/E, 58.6% of eyes) were strongly associated with enucleation (92.3% vs. 17.6%, p < 0.001) and proptosis (26.9% vs. 0%, p = 0.013). Conclusion: Early detection of retinoblastoma is crucial, as advanced-stage tumors, delayed presentation, and proptosis significantly increase the risk of enucleation in affected children.