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Wilms' tumour (nephroblastoma) is the most common abdominal malignancy in children. Extrarenal Wilms' tumour (ERWT) is rare, with limited reports in the literature. Hereby, we report a case of unilateral ovarian Wilms' tumour, which was diagnosed initially by closed biopsy and confirmed later by histopathology study of the excised tumour. We are discussing the unusual location and presentation of Wilms' tumour and showing the medical challenges in both the initial clinical impression and the pathological diagnosis. Demonstrating therapeutic plans and showing the good outcome achieved by using the classic renal Wilms’ tumour therapy protocols.

Keywords: Extrarenal tumour; Wilms' tumour; nephroblastoma; Ovary; paediatric tumour.

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How to Cite
Albiroty, K. A., Al Sabahi, A., Al Shabibi, S., Al’Ajmi, Z. I., Al Hinai, K., & Al-Mashaikhi, N. (2021). Extrarenal Wilms’ Tumour of the Ovary: A case report. Sultan Qaboos University Medical Journal [SQUMJ], 1(1).