In patients successfully treated for hereditary retinoblastoma, the risk of developing a second non-ocular tumor has been reported. We report the first case of primary hepatic leiomyosarcoma in a 39 year-old woman who has been treated 37 years before for hereditary retinoblastoma of the left eye. The patient presented with right upper quadrant abdominal pain and fever. Histological diagnosis was made by liver biopsy. As surgical resection was impossible, chemotherapy with epirubicin, then ifosfamide, etoposide and cisplatin was performed. The patient died 22 months after diagnosis. Genetic abnormalities observed in hereditary retinoblastoma, which probably resulted in a predisposition to the development of hepatic cancer in this patient, were not investigated.