The authors report a case of a 19-year-old man with alveolar rhabdomyosarcoma with an unusual clinical presentation. The diagnosis was established on lymph node biopsy performed on admission of the patient in a context simulating leukaemia. The patient achieved complete remission in response to high-dose chemotherapy. A recurrence in the form of an intratesticular metastasis demonstrated a periorbital tumour that was probably the primary tumour. The authors present the radiological and histological findings and a molecular biology study of the testicular metastasis, eliminating a primary testicular tumour.