We retrospectively studied the outcome in a consecutive series of 30 patients with desmoid tumors who were followed for more than 20 years after treatment (surgery in 29 patients). A local recurrence occurred in 12 patients and more than 1 recurrence occurred in 8. 3 patients had spontaneous complete regression of the tumor. At follow-up after mean 28 (20-54) years, all patients were tumor-free except 1 who had had a stable tumor for at least 11 years. Symptoms were related more to treatment than to the tumor. We conclude that desmoid tumors may have a high capacity for self-limitation and that conservative therapy should be considered in symptom-free patients.