RESUMO
INTRODUCTION: Vascular leiomyosarcoma is a conjunctive tumor which develops in the smooth muscle cells of vessel walls. Proximal vascular leiomyosarcoma involving the large vessels and peripheral vascular leiomyosarcoma which develops in the vascular pedicles of the limbs and subcutaneous vessels are distinguished. OBSERVATION: We report the case of a 6-year-old child who presented from birth a peripheral vascular leiomyosarcoma. The immunohistochimic study revealed tumor cell expression vimentin, SMA and desmine. In spite of wide surgical resection with associated chemotherapy, the child's clinical status deteriorated. DISCUSSION: Vascular leiomyosarcoma is a rare tumor occurring exceptionally in children. Prognosis depends on the presence of local recurrence and metastatic spread.