Purpose Iridociliary tumors are usually uncommon in childhood. Our aim is to describe cases of juvenile xanthogranuloma, iris tuberculoma and medulloepithelioma in young patients and to differentiate them from other similar lesions Methods Retrospective cases study. Four patients ( two girls and two boys) were referred at the Referral Center for Retinoblastoma of the University of Siena. They underwent all the ophthalmological examination including standard procedures, MRI of the orbits, CT, UBM, immunological and molecular analysis. In three cases organ‐preserving operations were performed. Results Two tumors were histologically identified as medulloepitheliomae. Two tumor‐like lesions were determined as juvenile xanthogranuloma and iris tuberculous granuloma. In the last case, antituberculous therapy was performed Conclusion Iridociliary tumors and pseudotumors are rather rare in childhood. Nevertheless, they should be taken in consideration in differential diagnosis with other pediatric intraocular tumors, particularly retinoblastoma, ciliary body adenoma and adenocarcinoma. An executive checkup including pathology, immunohistochemistry and immunology is to be performed.