Abstract Objective To study the epidemiology, clinical features, diagnosis, therapeutic management, and outcome of non-tuberculous mycobacterial lymphadenitis in a paediatric population of Aragon (Spain). Material and methods A retrospective study was conducted on patients under 15 years-old diagnosed with non-tuberculous mycobacterial lymphadenitis between the years 2000 and 2015. Inclusion criteria: patients with lymphadenitis and positive culture. Quantitative values are shown as mean, rank, and standard deviation, and qualitative data as frequencies. Results Twenty-seven cases were registered, with a mean age of presentation of 39.9 months (range 10 months–8 years). The mean time between the symptoms onset and first consultation was 1.7 ± 1.1 months. The most frequent location was sub-maxilar in 17/27 cases (63%), on the right side in 59.3%, and size 2.96 ± 1.26 cm. Fistulae were observed in 16/27 cases. Tuberculin test was greater than 10 mm in 7/24 (29.1%). Microbiological cultures were positive for Mycobacterium avium in 14/27 (51.9%), Mycobacterium intracellulare 3/27 (11.1%), and Mycobacterium lentiflavum 3/27 (11.1%). Combined treatment of antibiotics and surgery was given in 16/27 cases (59.8%), medical treatment only in 7/27 (25.9%), and surgical exeresis alone in 4/27 (14.8%). Two patients required a new surgery, and one showed severe neutropenia secondary to rifabutin. Only one case (3.7%) suffered from temporary facial palsy as sequel. Conclusions The most frequent treatment was the combination of antibiotics and surgery. Delay in diagnosis seemed to be responsible for the limited number of exeresis as first option, only one for every seven patients.