Purpose To explore the ocular manifestations associated with mutations in key tumor suppressor genes mutations (RB1, NF1, NF2, VHL, TSC1/2) in children, emphasizing their significance in pediatric hereditary cancer syndromes. These mutations are associated with common pediatric ocular tumors, presenting as space-occupying lesions in the retina and uveal tract (including the choroid and iris). Method A review of 11 representative case reports, complemented by an analysis of existing literature, was conducted to examine the ocular presentations linked to these mutations. The study focused on the genetic mechanisms underlying these conditions and their distinct ocular symptoms. Results Unique ocular manifestations were identified for each gene mutation, underscoring the critical role of regular and thorough ophthalmic monitoring. Early detection of these ocular symptoms enables timely intervention, improving both visual and overall patient outcomes. Conclusions Mutations in pediatric tumor suppressor genes are often associated with systemic syndromes. Recognizing ocular symptoms is vital for early diagnosis and effective management, helping to preserve vision and support developmental progress. Pediatric ophthalmic evaluations should be prioritized for early detection and comprehensive care. Future studies should aim to include more cases, employ diverse research methodologies, and develop long-term observation strategies tailored to specific tumor suppressor genes.