We describe an infant with a congenital polypoid anal mass, diagnosed as botryoid-type embryonal rhabdomyosarcoma. Histological examination revealed myxoma-like appearance and cells with greater differentiation with elongated streaming eosinophilic cytoplasm and one or two nuclei, suggestive of rhabdomyoblasts. Tumour cells were strongly immunoreactive for desmin and 10% of cells displayed immunoreactivity for myoglobin. We present this case with a view to highlighting the benign clinical and histological appearance of botryoid-type embryonal rhabdomyosarcoma. Early biopsy is essential for improving prognosis.