A 10-year-old girl with sclerosis tuberosa was admitted to the Children's Health Centre for febrile states of unclear origin. Ultrasonography revealed the presence of renal tumour of clear cell sarcoma character in histopathological examination. Despite nephrectomy the child died several weeks after the operation. The authors pay attention to the fact that both clear cell sarcoma and most lesions observed in sclerosis tuberosa have common origin from the nervous crest so their coexistence seems no accidental. In view of the observed in sclerosis tuberosa proneness to neoformation, the authors stress the necessity of periodical examinations of the brain and internal organs, mainly kidneys and liver.
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