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Background and Aim: Trilateral Retinoblastoma (TRB) is a rare, but well recognized syndrome that have a hereditary base. These tumors usually occur in the pineal, parasellar, or suprasellar regions, often several years after successful management of ocular retinoblastoma (RB) without evidence of direct extension or distant metastasis. The first report of TRB was in 1971 and fewer than 50 cases of TRB with suprasellar involvement have been reported in the literature. Case Report: Here we report a case of TRB with suprasellar involvement in a patient with bilateral retinoblastoma. The patient was a 2-year-old girl with red inflamed eye and vomiting during the last 15 days and lacrimation, leukocoria and proptosis from one week prior to admission. She was blind and deaf. Owing to special radiological signs, presence of calcification in the tumor, and limited number of differential diagnoses, diagnosis of TBR without pathological examination is possible. Conclusion: When TRB is detected in the brain CT-scan, It means that, the prognosis is very poor and survival rate will be less than 12 months. Because of poor prognosis of this syndrome, examination of the neonates and babies and checking the red reflexes and funduscopy in doubtful cases, at the time of birth are mandatory. Screening of the families of the patients with retinoblastoma and genetic counseling should be regarded necessary.