Introduction:Renal tumours account for over 6% of cancer diagnoses in children under 15 years old. Rarely they are diagnosed in newborns. Case report: A Female neonate born through a caesarean section in the 39 th week of pregnancy with palpable pathological dense mass located in the abdominal cavity in the physical examination of abdomen just after the labour diagnosed as a kidney tumour (ultrasound examination, MRI) was admitted to the Department of Paediatric Surgery for surgical treatment -right-sided nephrectomy and biopsy of regional lymph nodes. The histopathological examination revealed congenital mesoblastic nephroma (classical type). After consultation with the paediatric surgeon, pediatric oncologist it was decided that the girl did not require chemotherapeutic treatment. Conclusions: Congenital mesoblastic nephroma is one of the most frequent renal tumours in newborns and young infants. Most children with CMN have an excellent prognosis and are treated with nephrectomy with lymph node sampling.