Preiser's syndrome is a rare osteochondrosis affecting the carpal scaphoid, frequently related with an avascular necrosis. Osteoarthritic changes of the articular cartilage, local synovitis, and loose fragments are the most common findings associated with this syndrome. We report here two patients with Preiser's syndrome, one with and one without a traumatic history, both presenting with pain, swelling and functional impairment of the wrist. In one patient radiography was sufficient for the diagnosis, in the other NMR was necessary to clearly establish type and extension of the lesion. Differential diagnosis may be sometimes difficult and the therapeutic approach depends on several aspects, including etiology and type of occupational activity.