We describe a case of diffuse plane normolipemic xanthomatosis (DPNX) associated with Takayasu's disease and hyperhomocysteinemia. This report of an association of Takayasu's disease with hyperhomocysteinemia and DPNX is the only such report in literature. This report corroborates the study by Marcoval et al on 8 patients in 1988 where they highlighted that in every case of DPNX a possible concomitant condition should be always suspected and found out.