Nongastrointestinal mucosa-associated lymphoid tissue (MALT) lymphoma, although generally indolent in nature, has variable and heterogeneous clinical course and biologic behavior. We have evaluated the clinical features and follow-up of patients with MALT lymphomas involving the head and neck in our institution. Forty-four new patients with MALT lymphomas arising in ocular adnexa (24 patients, group A) and other head and neck sites (20 patients, group B) of the salivary gland (13), thyroid gland (3) and other regions (4) were retrospectively analyzed. At initial diagnosis, 21 patients had solitary lesions (16/24 in the group A vs. 5/20 in the group B, P=.008); 23 had disseminated disease at multiple sites (11 patients) and/or lymph node involvement (15 patients: 3/24 in the group A vs. 12/20 in the group B, P=.025). Treatment consisted of local therapy, consisting of surgical resection and/or radiotherapy, in 34 patients and systemic chemotherapy with/without local therapy in 10 patients. Eight patients experienced tumor recurrence: four patients died of disease, two were alive with disease, and two were disease-free at last follow-up. With a mean follow-up of 46 months, the 5-year overall survival and relapse-free rates were 83% and 74%, respectively, which did not differ between the two groups (P>.5). Patients with MALT lymphomas of the head and neck are at relatively high risk for multifocal or lymph node involvement. Recurrence at prolonged times after therapy indicates the need for close long-term monitoring, and for prospective trials to prevent recurrence.