Rhabdomyosarcoma is an uncommon tumour that may present at a wide vari
ety of different sites. We report a 4-year-old girl who developed an e
mbryonal rhabdomyosarcoma arising in the left pinna which was clinical
ly indistinguishable from a lymphangioma. The case illustrates that th
is neoplasm can be easily misdiagnosed because of its variable morphol
ogy. Early recognition is important as successful treatment is now pos
sible with a combination of chemotherapy, surgery and/or radiotherapy.