A case study of desmoplastic small round cell tumor with divergent differentiation in a 13-yr-old boy is presented. Clinical, cytologic, histologic, immunohistochemical, and ultrastructural findings are reported. There was a close correlation between the various histologic and cytologic patterns. Immunohistochemical studies revealed strong positivity of many of the tumor cells for cytokeratin, vimentin, and desmin. Ultrastructurally, some of the tumor cells contained aggregates of cytoplasmic intermediate filaments. Fine-needle aspiration biopsy may suggest the diagnosis of desmoplastic small round cell tumor. Confirmation of the diagnosis may be provided by immunohistochemistry and electromicroscopy.