Herein, we present a case of aortic root replacement 42 years after aortic valve replacement (AVR) with the Björk-Shiley Spherical (BSS) valve in a patient with an aortic root aneurysm. The patient was a 67-year-old man who had undergone AVR with BSS and aortic root enlargement for the treatment of infective endocarditis and aortic insufficiency at 25 years of age. He underwent aortic root replacement for an enlarged aortic root (73 mm). Under general anesthesia, median re-sternotomy was performed, and the BSS valve was removed. The valve functioned well with no pannus or thrombus. We performed an aortic root replacement using a composite graft consisting of a 24-mm mechanical valve and 30-mm artificial graft. We experienced a rare case of long-term durability of the BSS valve, which functioned well for 42 years.