Despite the increasing prevalence of cervicofacial lymphadenitis due to atypical mycobacteria (AMB) in children, the true nature of AMB infection in clinical practice is poorly understood. The purpose of our study was to define the most common signs and symptoms, and to establish a workable scheme of diagnosis and treatment. Patients fulfilling the criteria of AMB infection (i.e., clinical signs, positive cultures or polymerase chain reaction, histologic features) were included in the study. All children underwent a standard surgical procedure, depending on pretreatment and the course of the disease. Sixteen infants presented with characteristic unilateral lymphadenopathy predominantly involving the submandibular area (13/16). Eight children had been initially treated at various institutions by fine-needle puncture or incision, and 7 of the 16 patients had received antituberculous multidrug treatment for a varying length of time. Complete excision of the affected lymph nodes was the definitive treatment in all patients. Three children had transient marginal mandibular nerve paralysis that resolved within a few months in all cases. Recognition of the characteristic features of AMB adenitis may permit early diagnosis and appropriate surgical treatment.