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Abstract Desmoid tumours are a rare type of tumours. Retroperitoneal desmo id is an uncommon mesenchymal neoplasm presenting as a firm mass with locally aggressive features. Desmoid tumour account for 0.03% of all tumours and 3% of all the soft tissue tumours. It usually manifests as a well-circumscribed or ill-defined, solid mass on cross-sectional imaging. The confirmatory diagnosis of these tumours is made by histological examination and immunohistochemistry. Surgical resection of the tumour is the treatment of choice in Desmoid tumours and also helps in clearing the diagnostic dilemma it creates in patient because of its rare nature. Here, we present a rare case of a Large Retroperitoneal Desmoid-type Fibromatosis of renal origin in a 28-year-old female, which was successfully treated surgically at specialised oncology centre in India.