Calcifying nested stromal-epithelial tumour (CNSET) of the liver is a rare tumour, recently included in the WHO Classification of Digestive system Tumours 2019. Its hallmark is the nested proliferation of epithelial and spindle cells with surrounding desmoplastic stroma, calcifications and ossifications. Less than 40 cases have been reported, whereas none has been reported in Middle Eastern descendants. We report the first case of CNSET in a 22-year-old Egyptian lady with detailed histological and clinical data.