The Rationale: Rhabdomyosarcoma (RMS) is an uncommon tumour which presents at a wide variety of different head and neck sites. They are highly aggressive malignant mesenchymatous soft tissue sarcomas. Their occurrence in the outer ear is extremely rare. Patient Concerns: A 17-year-old female patient reported with a chief complaint of steady increase in growth over the left ear since 6 months, which is not associated with pain and ulceration. Diagnosis: Edge biopsy sections of the swelling over the left pinna and immunohistochemistry records of cells show MYODI positive confirming as Embryonal RMS. Treatment: Left auriculectomy and supra-omohyoid neck dissection with level V lymph node was performed. Outcomes: Uneventful postoperative healing in the resected site was elicited in the follow-up period of 1 year with no recurrence. Take-away Lessons: External ear RMS is extremely rare. Careful clinical, radiological and histopathological evaluation are essential for prompt definitive diagnosis and for successful management.