Abstract:AIM: To describe the clinicopathologic features and classification of pediatric and adolescent ocular tumors and tumor-like lesions METHODS: A total of 719 cases of pathologically confirmed ocular tumors and tumor-like lesions in a pediatric population from two academic institutions over an 18-year period were retrospectively analyzed. The main outcome measures were the clinical and pathological features of the cases. RESULTS: Benign tumors accounted for 92.1% of all cases while malignant tumors accounted for 7.9%. The most common ocular benign tumors were (epi-)dermoid cysts (19.8%), nevi (15.2%), corneal dermoid tumors (9.8%), and calcified epitheliomas (8.8%). The most common ocular malignant tumors were retinoblastoma (80.8%), and rhabdomyosarcoma (3.9%). Eyelid and ocular surface tumors comprised 73.3% of benign tumors while intraocular and orbital cavity comprised 94.2% of malignant tumors. For tumor site, the upper eyelid was up to 1.79 times more than lower eyelid (P<0.05). Age at surgery and sex also had an association with different lesions (P=0.006, P=0.035, respectively). CONCLUSION: Most ocular tumors and tumor-like lesions in children and adolescents are benign. Pediatric ocular tumors are distinct from those in adults in terms of histological origin. (Epi-)dermoid cysts are the most common benign tumors while retinoblastomas the most common malignant tumors.