Rhabdoid tumour of the kidney (RTK) is considered to be one of the most aggressive neoplasms of early life. The histogenesis of RTK still remains a matter of controversy. Immunohistochemistry usually shows diffuse reactivity for vimentin, focal reactivity to the epithelial marker, variable expression of mesenchymal and neuroectodermal markers, and loss of INI1 protein staining. Expression of the Wilms’ tumour protein (WT1) was described in the RTK cases. We would like to present a case of rhabdoid tumour of the kidney in Latvia, which caused diagnostic difficulties of a 27-month-old girl, and a short review of literature.