Rhabdomyosarcoma is one of the commonest soft tissue sarcomas of childhood, but neonatal presentation is extremely rare. This limited experience means there are no clear treatment guidelines in this age group. The authors report a boy with recurrent attacks of hematuria commencing in the neonatal period, which were shown to be from a prostatic rhabdomyosarcoma. To the best of our knowledge this is the first reported case of neonatal rhabdomyosarcoma in this site. © 2004 Wiley-Liss, Inc.