Myopericytoma belongs to a family of benign tumors that exhibit a myoid/pericvtic line of differentiation.1 The most common anatomical sites for myopericytoma are the skin and superficial soft tissues of the distal extremities.2 With increased clinical recognition, a wider distribution has been described;3-5 however, a pulmonary lesion has never been reported. We now report a case of primary pulmonary myopericytoma and review the literature on this rare entity.