Patients with primary amyloidosis (AL) have poor prognosis with a median survival of one to two years. In patients with symptomatic cardiac involvement, prognosis is even worse with the reported median survival of 6 months. One patient, diagnosed as AL amyloidosis with cardiac involvement, was treated with thalidomide and showed remarkable improvement in functional capacity, cardiac function, and various laboratory parameters without any significant adverse effect and, therefore, we report the case with the review of literatures.