Myxoid adrenocorticfal adenomas are extremely rare with only 24 cases reported.1-12 The first case was reported by Tang et al1 in 1979 which was diagnosed as a myxoid adrenocortical carcinoma. Nearly all the reports were of single case except Browen et al3 who described a group of 14 cases. We report here an additional case of myxoid adrenocortical adenoma with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.