Desmoplastic small round cell tumour (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects adolescent male and young adults and most frequently presents as a large abdominal mass with widespread peritoneal involvement at the time of diagnosis. Histologically, it is composed of nests of small, undifferentiated round or oval hyperchromatic cells embedded in abundant desmoplastic stroma. Co-expression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence of origin from a primitive pluripotent stem cell with multiphenotypic differentiation. A multidisciplinary treatment including high-dose chemotherapy, aggressive debulking surgery, radiation and myeloablative chemotherapy with stem cell rescue might be the proper approach to treat this rare malignancy and may improve progression-free survival.