The authors report a case of an intrapulmonary tumour which on histolo
gical analysis was found to be a malignant Triton tumour after surgica
l excision. The tumours proved to be of nervous tissue origin and was
a rare variant of malignant tumours of the surrounding of peripheral n
erves with rhabdomyoblastic heterologous differentiation which is rare
ly described in the lungs. The characteristics of the tumour as well a
s therapeutic approaches are discussed followed by a review of the lit
erature. A particular aspect of the case presented was to realise that
there was a right to left shunt and this is also discussed.