A diagnosis of mesenchymal tumor with DICER1 alteration should always be taken into consideration when dealing with a mesenchymal neoplasm with areas of a subepithelial layer of malignant mesenchymal cells, areas of rhabdomyoblastic differentiation, cellular/immature and sometimes overtly malignant cartilage or foci of bone/osteoid. This evidence concerns the gene DICER1 and mesenchymal cell neoplasm.