Seventeen pediatric patients with a major salivary gland malignancy (16 parotid, 1 submaxillary) were reviewed. Eight patients presented with carcinoma. The usual presentation was a mass over the affected gland. Six patients had localized disease, which was treated by excision. This was accomplished by either a total or subtotal parotidectomy or resection of the submaxillary gland. Two patients received adjuvant radiation therapy. All six patients with localized carcinoma are alive, without evidence of disease. Two patients presented with metastatic disease and died of the disease despite treatment with multiagent chemotherapy, and in one case, radiation therapy. Nine patients had rhabdomyosarcoma (RMS). The usual presentation was a mass at the angle of the mandible. Five patients had involvement of one or more cranial nerves, and two had concomitant cervical adenopathy. Eight patients had a biopsy and then were treated according to an existing prospective institutional protocol. The ninth patient initially underwent a superficial parotidectomy. Seven patients received radiation therapy. In one patient, rapid progression of the disease precluded this treatment. Seven patients died of progressive local and distant disease 2 months to 2 years (median, 6 months) from the time of diagnosis. Two patients are alive, without evidence of disease, 3 and 7 years after presentation. We conclude that carcinoma should be managed with complete excision. For RMS of the salivary gland, a biopsy should be performed, and treatment should consist of chemotherapy and radiation therapy. © 1994.