Background: In the Netherlands a comprehensive program for screening just after birth for familial retinoblastoma is taking place. In this report we evaluate the stage of the disease at the time of detection, by way of screening, and the long term visual outcome in these patients. Materials and methods: A nation wide, retrospective study. From January 1992 - July 2004, patients at risk for familial retinoblastoma were screened 1-2 weeks after birth, and investigated for laterality, Reese-Ellsworth classification/ International Classification of Retinoblastoma, macular involvement, age of primary RB, initial therapy, and visual outcome. Results: 17 patients were diagnosed with familial retinoblastoma. 88.3% developed bilateral, 11.7% unilateral retinoblastoma. Of the 34 eyes, 56% were R-E Group I, 16% were Group II A-B, 16% were Group III A-B, 9% were Group IV, 3% were Group V. Using the International Classification of Retinoblastoma, 72% were group A, 19% were Group B, 6% were Group C, 3% were Group E. The visual outcome revealed 73.5% of eyes with 20/20-20/40, 26.5% eyes with 20/100-no light perception; 5.9% of eyes were enucleated, all other eyes were treated with local or conservative treatment methods. Of all eyes, 59% had extra macular RB, 98% of patients had at least one eye with extra macular RB. Conclusion: Most familial retinoblastoma patients present as a R-E Group I or group A when screened within 2 weeks after birth. Nearly 90% of patients had a long term visual acuity of 20/20-20/40. Despite the common occurrence of macula involvement, bilateral macula involvement was infrequent and since most eyes were salvaged good vision was obtained in the majority of patients.