Mediastinal bronchogenic cysts are usually asymptomatic being often diagnosed in young adults. We present a case of a 2-year-old boy in severe respiratory distress due to a large mediastinal cyst that underwent urgent ultrasound-guided percutaneous decompression followed by planned strictly thoracoscopic excision. This unique report highlights that bronchogenic cysts may develop acute compressive symptoms requiring prompt drainage, which enables elective resection; and that both procecedures can be accomplished successfully by fully minimally invasive techniques.