Epithelioid sarcoma is a very infrequent soft tissue sarcoma involving predominantly distal extremities of adolescent and young adult. We hereby report a case of epithelioid sarcoma in a 34-year-old young adult who presented with 1-year history of a painful left upper limb associated subsequently with warm left forearm swelling and a Volkman contracture. He was treated as an inflamed soft tissue condition of the left upper limb. A computed tomography study showed presence of multiple hypodense lesions mainly in the flexor compartment of the left arm as well at the subcutaneous tissue, which measured 1 to 1.7 cm in diameter. Histological examination of the left upper limb mass showed nodular proliferation of epithelioid tumour cells and some with rhabdoid features surrounding a central zone of necrosis and was diagnosed as epithelioid sarcoma. Concurrent presence of epithelioid sarcoma and Volkman ischaemic contracture are rarely seen in clinical practice. The present case highlights the importance of the histology which can be confused with other types of sarcoma or chronic granuloma and even missed at times thereby causing diagnostic problems.