We report a case of sarcoma botryoides of the uterine cervix occurring in a 19-year-old woman. By light microscopy the tumor showed round and spindle cells with hyperchromatic nuclei and, focally, a cambium layer subjacent to the surface epithelium and surrounding endocervical glands. Strap-shaped cells with and without cross-striations and small foci of immature cartilage were also present. Immunohistochemical studies showed positive staining within the tumor cells for myoglobin, desmin, vimentin, muscle-specific actin and CD56. By electron microscopy, tumor cells showed cytoplasmic filaments in an alternating pattern of thick and thin filaments. Chromosomal analysis demonstrated deletion of the short arm of chromosome 1, and trisomies 13 and 18. To our knowledge, this is the first reported case of sarcoma botryoides of the endocervix with chromosomal analysis.