CONTEXT: Carney complex (CNC), a familial multiple neoplasm syndrome with 
dominant autosomal transmission, is characterized by tumors of the heart, skin, 
endocrine and peripheral nervous system, and also cutaneous lentiginosis. This 
is a rare syndrome and its main endocrine manifestation, primary pigmented 
nodular adrenal disease (PPNAD), is an uncommon cause of adrenocorticotropic 
hormone-independent Cushing's syndrome.
CASE REPORT: We report the case of a 20-year-old patient with a history of 
weight gain, hirsutism, acne, secondary amenorrhea and facial lentiginosis. 
Following the diagnosing of CNC and PPNAD, the patient underwent laparoscopic 
bilateral adrenalectomy, and she evolved with decreasing hypercortisolism. 
Screening was also performed for other tumors related to this syndrome. The 
diagnostic criteria, screening and follow-up for patients and affected family 
members are discussed.