Primitive neuroectodermal tumors of the hand are extremely rare, and only 5 cases have been described to date. Here, we report a case of a 35 year-old male who presented a progressive swelling on the palm of his right hand. Clinical examination showed a solid mass and X-ray revealed a soft tissue mass. Magnetic resonance imaging (MRI) revealed infiltrated interosseous muscles, metacarpal bones and tendons. The patient underwent surgery and the lesion was removed. On the basis of morphological, immunohistochemical and molecular biology findings, a diagnosis of primitive neuroctodermal tumor was made.