With a follow up of 14 years, a benign intra nodal palissading smooth muscle tumor is reported as an original lesion because of the expression of HMB 45. This antibody is now well known to be expressed by non melanocytic smooth muscle lesions, like angiomyolipoma, "sugar tumors" of the lung, pulmonary lymphangiomyomatosis and some others, often linked to tuberous sclerosis. The case reported seems to be a new member of the merging concept of "Perivascular Epithelioid Cells (PEC)".