Retinoblastoma (RB) is the most common intraocular tumor in the childhood. Commonest presentation of RB is leukocoria. In pathology, gross examination, histopathology, molecular pathology and genetics plays an important role. Pathological staging and AJCC 8th staging of RB are important while reporting the pathological samples. Often differential diagnoses of RB pose a problem in clinical and pathological diagnosis.