Background. Echinococcus disease in still endemic in many low-middle-income countries, with 1 million people infected at any one time. Surgery, the mainstay of its treatment, is costly. Objectives. To describe the presentation, clinical features and outcomes of children referred with pulmonary hydatid disease at Inkosi Albert Luthuli Central Hospital in Durban, South Africa. Methods. A 10-year review of children with pulmonary hydatid disease at Inkosi Albert Luthuli Hospital was carried out. The data collected were demographic, clinical, laboratory and radiological. Cases were mapped geographically to analyse for clustering of cases. Spearman's correlation was used to assess for correlations between laboratory markers. Results. A total of 24 subjects, 75% of whom were male, were included. The mean (standard deviation) age at diagnosis was 8.9 (3.4) years. The mean delay in diagnosis was 5.8 (5.7) months. Of the subjects, 15 (71.4%) were from the Eastern Cape and 9 from KwaZulu-Natal provinces. Seventy-nine percent of the patients had been exposed to dogs, while 8% reported exposure to either sheep or cattle. There was right-sided preponderance, with 11 right-and 7 left-sided cysts; 6 patients had bilateral cysts, and 4 associated extrapulmonary cysts. Indirect haemagglutination assay was positive in 70%, and blood eosinophilia was present in 45% of the subjects, with no correlation between the two markers (p=0.22). Surgery was the only modality of treatment in 18 (75%) subjects, while 5 had had prior medical therapy for disseminated disease. The mean intensive care stay postoperatively was 2 (2) days with no mortality. Conclusion. Despite exposure to known risk factors and living in endemic regions, there is often a significant delay in diagnosis of pulmonary hydatid disease at Inkosi Albert Luthuli Hospital.