The article addresses the issue of a combination and relationship between intramedullary spinal cord tumors (IMSCTs) and neurofibromatosis (NF). Objective. To study, based on large clinical material, features of the prevalence and biological nature of intramedullary spinal cord tumors in neurofibromatosis patients in different age groups. Material and methods. We analyzed the data of the largest series of patients from all age groups who underwent surgery for spinal cord intramedullary tumors (541 patients; 586 surgeries; age, 2 months to 72 years). Results. Our findings support a potential pathogenetic relationship between intramedullary tumors and neurofibromatosis. Astrocytoma was a predominant intramedullary tumor in patients with NF-1, while ependymoma predominated in patients with NF-2. IMSCTs combined with NF-1 occur predominantly in children and adolescents, while a combination with NF-2 is typical of young adults. Our findings confirm the fact that IMSCT surgery is required for a small number of NF patients.