SUMMARY. Primary spontaneous pneumothorax (PSP) is defined as pneumothorax occuring in the absence of trauma or underlying lung disease. PSP is rare in childhood and occurs mainly in two age groups, neonates and adolescents. Purpose: Three cases of PSP treated in 2006 are presented, with a literature review. Method: Analysis of patients’ medical records and a short review of the relevant literature. Results: The patients were a 2 day-old female neonate and two adolescent boys aged 12 and 16 years respectively. The adolescent boys both had tall, thin stature with low body mass indices for their age. All the patients were treated with tube thoracostomy, which was removed after a mean of 3.6 days. In one of the adolescents high-resolution CT was normal, although examination of two male relatives revealed subpleural apical blebs and bullae (familial PSP). No recurrences were observed in any of the patients during hospital stay or follow-up for a mean of 1.1 year. Conclusions: PSP is almost always associated with subclinical emphysema-like lesions (blebs and bullae) in the apical regions of the lung. In children, PSP usually occurs in adolescence, affecting mainly tall, thin boys, but it is also seen in neonates, especially premature infants. The disease is usually sporadic, but familial cases account for up to 10%. PSP is usually treated with tube thoracostomy. More invasive methods such as thoracotomy or thoracoscopy with excision of the involved lung parenchyma and pleurodesis aim at preventing or treating PSP recurrences, which are encountered frequently (16-52%). Pneumon 2008; 21(1):52–59