Retinoblastoma is the commonest childhood primary malignant intraocular neoplasm that is often characterized by spontaneous regression. They display photoreceptor differentiation. This study provides the clinical presentations and histological profiles of retinoblastoma in Ilorin, Kwara – State, in the North Central geo-political zone of Nigeria. A retrospective study of clinically and histologically verified retinoblastoma at the University of Ilorin Teaching Hospital, Ilorin, Kwara – State, Nigeria from January 1989 to December 2000 was undertaken. The clinical and histological features were analyzed using the patient's case folder and surgical pathology records. There were 20 patients, 9 males and 11 females (M:F ratio 1: 1.2), age range from 5½ months to 6 years with 23 eyeball tumours histologically confirmed retinoblastoma during the study period. Proptosis with chemosis was the most common clinical presentation (84.6%). Bilaterality was 15% in this study. Enucleation and Exenteration combined with chemotherapy were offered to 15 (75%) and 5 (25%) patients respectively. A poorly differentiated type with extensive areas of tumour necrosis was the commonest histological pattern. Thirteen (65%) of the patients died before completing the course of chemotherapy. African Journal of Health Sciences Vol. 12(3-4) 2005: 94-100