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To estimate the incidence and distribution of nontuberculuous mycobacterial surgery, a retrospective case record study of 42 children operated for cervicofacial atypical mycobacterial infections in Oslo from 1990 to 2000 was performed. Mean age at diagnosis was 41 months and mean duration of the disease was 40 weeks from symptom onset to remission. All presented with localized and unilateral disease without increased hematological parameters. The majority of children had one lesion localized in the submandibular region characterized by changes in the overlying skin color, but without necrosis or fistula formation. Children below three significantly more often presented with only one lesion and the referring physician more frequently suspected neoplasm and bacterial adenitis than reactive adenopathy in this group. Compared to older children, there was a tendency for shorter symptom duration prior to outpatient treatment (mean 4 and 13 weeks for children up to 3 and above 3 years, respectively, t=-1.6, P=0.11). Furthermore, mycobacterial cultures and histopathology from surgical specimens was needed to diagnose the disease correctly. Fine-needle aspiration biopsy was unsuccessful in 27% (n=8) of the patients, due to non-cooperative patients (n=2) or inconclusive material (n=6), and mycobacterial growth was not obtained in any of the samples. Intradermal mycobacteria skin testing yielded 29% (n=10) false negatives. Although mycobacteria was correctly diagnosed in the remaining patients, correct specimen was found in only 31% (n=11) of the 25 cases.