Background: Retinoblastoma is a common malignant intraocular tumor of early childhood that is potentially vision- and life-threatening, with outcomes strongly influenced by timeliness of diagnosis and access to appropriate multimodal treatment. The purpose of the study is to evaluate the clinical presentation, diagnostic challenges, treatment modalities, and therapeutic outcomes in pediatric patients with retinoblastoma. Methods: This hospital-based descriptive study was conducted at the Department of Ophthalmology, National Institute of Ophthalmology and Hospital, Dhaka, Bangladesh, from April 2011 to March 2013, and included 46 pediatric patients with retinoblastoma. Clinical presentation, diagnostic features, disease staging, treatment modalities, therapeutic response, and complications were evaluated using comprehensive ophthalmic examination and imaging. Treatment was individualized according to disease severity, and patients were followed to assess outcomes. Data were analyzed using descriptive statistics. Results: Among 46 children, most were ≤3 years at diagnosis (73.9%; mean age 2.5 ± 1.6 years), with male predominance (56.5%). Unilateral disease occurred in 71.7%, and leukocoria was the commonest presentation (69.6%). Nearly half had delayed presentation (45.7%). Most presented with Stage II disease (60.9%). Enucleation with radiotherapy was the most frequent treatment (26.1%). Disease control was achieved in 89.1%, with metastasis in 6.5% and mortality in 4.3%. Neutropenia was the commonest complication (17.4%). Conclusion: Early recognition and timely, multimodal treatment of pediatric retinoblastoma are crucial for effective disease control and favorable therapeutic outcomes.