SUMMARY
Intraocular tumors in childhood need precise diagnosis. In the present work we report a case of intraocular tumor in an infant with 33 days of age, who had a previous diagnosis of retinoblastoma. The patient was refered to the pediatric service due to the presence of respiratory problems. The clinical and auxiliary exams showed various cardiac tumors. We needed for performing a retinal biopsy to elucidate the case. The biopsy was performed through pars plana vitrectomy and the histopatologic examination revealed an astrocytic hamartoma of the retina, compatible with tuberous sclerosis.