Introduction: Retinoblastoma is one of the pediatric malignancies and its treatment is still challenging. The objective of this study was to highlight the sociodemographic, clinical, and therapeutic aspects of retinoblastoma at the Donka Paediatric Haematology/Oncology unit in Donka university hospital, Guinea, and thereby highlight the conditions of this malignancy in this country. Methods: This was a retrospective study over 3 years (January 1, 2017 to December 31, 2019). The study population was all retinoblastoma patients who were treated in this unit. Socio-demographic, clinical, and therapeutic data were analyzed. Results: Out of 210 pediatric patients followed up for cancer in this unit during the period, retinoblastoma accounted for 46 (22%). The patient age at diagnosis was 35.17 months (range: 3-132 months). At admission, metastases were observed in 16 patients (35%): 14 with brain metastases and 2 with lymph node metastases. Preoperative chemotherapy was employed in 38 (83%), tumor resection was performed in 20 patients (43%), and postoperative chemotherapy was performed in 19 patients. As for the prognosis, remission in 9, death in 14, and treatment discontinuation in 15. Only one patient had health insurance (2%). Conclusion: In this region of Guinea, retinoblastoma is the most common ocular tumor in children. Its diagnosis is easy: strabismus and leukocoria are the early signs of retinoblastoma. The prognosis depends on how early the diagnosis is made.