Abstract
Epithelioid sarcoma (ES) is a rare soft tissue tumor that is commonly misdiagnosed. It is a mesenchymal tumor that shows both mesenchymal and epithelial features. It tends to occur in the distal upper extremity in children and young adults but may appear in any location and any age group. Less than 1% of ES involve the head and neck. Clinically, the tumor can be mistakenly confused with a benign lesion as it can mimic nonspecific ulcers or infected warts. Histologically, ES is characterized by a nodular architecture and epithelioid appearance of cells centered with necrosis, mimicking a granulomatous process. We present the clinical history of a 12-year-old male who presented with an ES of the pretragus with a brief review of the literature to raise awareness on this rare entity and to discuss the challenges in managing histopathological differential diagnosis in front of this unusual clinical presentation.