Epithelioid sarcoma is an infrequent tumour, especially in upper limb, which nerve spread is not common. We describe the case of a 27 years old caucasian male with initial diagnosis of Dupuytren's disease. He underwent palmar fascia surgery, but the surgical treatment was followed by recurrence of the deformity in retraction of 4th and 5th finger of the left hand. When he presented himself at our attention, the patient underwent surgical biopsy and new histological examination was made with diagnosis of epithelioid sarcoma. We highlight essentially two aspects of this case: the diagnostic delay of tumour caused by simulation of the Dupuytren's disease and the atypical perineural spread along the median nerve.