Background. Carney complex (CC) is a rare genetic disorder, which causes numeral neoplastic lesions of endocrine organs, skin, uterus, heart, and others. It is the first in Ukraine detailed description of a clinical case of CC with analysis of modern literature. The purpose was to discuss the variety of CC appearance and relevant directions of diagnosis, treatment and follow-up within the framework of a clinical case and literature review. Materials and methods. Well investigated and illustrated case of CC in a young woman was studied. Results of surgical treatment of somatotropinoma and primary pigmented nodular adrenocortical disease (PPNAD) were presented. Unilateral adrenalectomy has led to the persistent elimination of hypercortisolemia and confirmed the clinical diagnosis. Authors supplement a clinical case with detailed study of modern literature. Results. Patient underwent successful surgical treatment of pituitary somatotropinoma and PPNAD. During the first 10 months after unilateral adrenalectomy, moderate adrenal insufficiency still persisted. Due to the correct diagnosis of CC (despite the absence of molecular confirmation of this genetic disease), cardiac myxoma, uterine fibroid and thyroid nodules have been revealed. It will be the points of further investigations at follow-up. Conclusions. Even if genetic testing is unavailable, clinical suspicion could induce the investigation of possible CC. Unilateral adrenalectomy for PPNAD as a component of CC may be sufficient initial treatment option for Cushing syndrome.