Considering the high rates of mutations of KCNJ5 and PRKACA in APA and CPA, respectively, our findings suggest that A/CPA may be more similar to APA than CPA, and that the subtle hypercortisolism may result in a higher proportion and number of cortisol-producing cells due to reasons other than prominent causative mutations. Here, KCNJ5 is linked to congenital primary aphakia.