FH is further subclassified into types I through IV according to different gene mutations.<h4>Case presentation</h4>This paper reports a case of unilateral adrenal hyperplasia with germline CACNA1H mutation, p.His515Tyr and p.Tyr613Phe, confirmed by endocrine test, whole exome sequencing and Sanger sequencing 4 years after onset. Here, CACNA1H is linked to familial hyperaldosteronism.