Background: Retinoblastoma, although rare, is the most frequently occurring primary intraocular tumor of childhood. While a cure is often achieved where appropriate treatment is instituted early in the disease, late presentation often leads to unsuccessful treatment interventions. Aim: The aim of this study is to provide baseline information on the clinical presentation of retinoblastoma as seen in a tertiary pediatric eye care facility in Calabar, Nigeria. Materials and Methods: A retrospective review of case files of children presenting to the Calabar Children's Eye Center (CCEC) with retinoblastoma over 18 months from January 1, 2018 to June 30, 2019 was done. Results: Data were collected from 39 children with retinoblastoma who met the inclusion criteria. There were 22 (56.4%) males and 17 (43.6%) females, and the age range was 7–41 months, with a mean age of 25.6 (±9.0) months. Unilateral disease was seen in 27 (69.2%) participants, whereas 12 (30.8%) had bilateral disease. Although multiple symptoms were seen in many participants protrusion of eye was the most frequently occurring first symptom, (17, 44.0%) followed by red eye (13, 33.0%), whitespot in eye (5, 13.0%), and abnormal alignment (4, 10.0%) among study participants. The lag time from recognition of symptoms to initial consultation ranged from 1 to 25 months, with a mean lag time of 9.13 ± 5.68 months. The ICRB classification system was most frequently used, 27 (69.2%), followed by cTNMH 11 (28.2%), and International Intraocular Retinoblastoma Classification 1 (2.6%). The International Retinoblastoma Staging System was representative. Of the 78 eyes studied, (51, 65.3%) had different stages of retinoblastoma with the majority presenting as advanced retinoblastoma (46, 90.2%). Conclusions: Retinoblastoma is seen at the CCEC. Although the most frequent symptom was protrusion of eye, the lag time was often several months long. Most patients presented late with advanced ocular retinoblastoma and or extraocular disease.