Introduction: Retinoblastoma is a malignant tumour occuring among the children up to three years old. Despite a good prognosis, an early prevention and recognition of disease signs are very important. The role of the nurse through prevention programmes and monitoring the child from the discovery of the disease to rehabilitation and further is therefore essential. Purpose: The purpose is to describe the role of a nurse in treating for a child with retinoblastoma. Methods: We used descriptive working method along with a critical review of domestic and foreign scientific literature. We searched different databases, CINAHL, MedLine (Pubmed) and cooperative bibliographic system COBISS.SI. Discussion and conclussion: A nurse has an important role in treatment of children with retinoblasoma. The role of nursing is extremely important from the first stage of early detection of the disease. Through health-educational work parents and others are trained to detect early warning signs of retinoblastoma. The role of the nurse becomes even more visible when a child gets sick – the nurse spends most of the time with the child and his parents. As an expert whose treatment of a child is integrated, she knows that her work continues after the hospital discharge, in the rehabilitation period and later in further monitoring of the child’s health status. The nurse is an important part of the interdisciplinary team, which is responsible for a good prognosis in the treatment of a child with retinomblastoma. In the field of prevention there are still a lot of opportunities for nurses to be even more involved.