Ewing sarcoma family of tumors (EFTs) of the head and neck are rare and account for only 1 – 4% of all EFTs. Here, we describe an extremely rare case of Ewing’s sarcoma/PNET arising from middle-external ear in a 32-year old female, who presented with growth from external auditory canal. As per my knowledge, this is the first case reported in India. This patient is taken on EFT 2001 protocol and is doing well. Aggressive multimodality treatment including chemotherapy and surgery/radiation can improve the survival of these patients.