Intrathoracic lipoma is a comparatively rare disease. We have recently experienced a case of intrathoracic chest wall type lipoma associated with Charcot-Marie-Tooth disease. The case was a 16-year-old man, in whom preoperative examination of his foot-drop due to Charcot-Marie-Tooth disease pointed out an abnormal chest shadow. Image diagnosis led to intrathoracic chest wall type lipoma. The tumor was easily resectable and was lipoma pathohistologically as well. The literature presented us with 22 cases of such lipoma, which appeared to be predominant for age not more than 10 and age 41-60 and to occur favorably in postero-superior chest wall. Preoperative diagnosis of the present disease became possible to a certain extent by means of CT, MRI. However, in view of imperfect differentiation between lipoma and liposarcoma, surgical resection should be considered, if possibly. No case of intrathoracic lipoma associated with Charcot-Marie-Tooth disease had been reported in the literature, and the causal relationship there between also remains unknown, but to be further examined.