We described a case of an endogenous hypercorticism in 18-years old patient with Itsenko - Cushing syndrome which was caused by a hormonally active adrenal tumor of the left adrenal gland. In a clinical picture of a disease the following symptoms dominated: arterial hypertension, increase in body weight, adiposity of the trunk, hands, neck, face, strips of extensions of skin on femurs. We found multiple nevus pigmentosus of black, yellowy-brown color from 1.5 to 10 mm on lips and mucous of a mouth, skin of a face and a trunk. The hormonal research established violations of a circadian rhythm of a hydrocortisone, increase in the free hydrocortisone of blood to 749.0 nmol/24 h (reference range 123.0-626.0), urine to 1314.0 nmol/24 h (reference range 60.0-413.0). The ACTH level decreased to 1.7 pg/ml (reference range 7.0-66.0). The negative result of the night overwhelming test with Dexamethazonum is received. Muitislice CT of adrenal glands revealed signs of a nodular hyperplasia of the left-hand adrenal gland. The laparoscopic adrenalectomy is made at the left. Pathoanathomical conclusion: a nodular pigmental hyperplasia of a glomerular and fascicular zone of bark of an adrenal gland with inclusion of a miyelolipomatoz. In the nodular corticocytes inclusions of lipofuscin granules were found. In the postoperative period symptoms of a hypercorticoidism regressed, throughout two years of observation there is no recurrence. Monitoring of hormones of blood in six months: hydrocortisone 359.5 nmol/l, ACTH 7.02 pg/ml. Retrospectively, the patient displays an infrequent heritable disease - Carney complex. The patient's mother at the age of 32 years underwent left-side adrenalectomy concerning Itsenko - Cushing disease with morphological confirmation of a nodular pigmental hyperplasia in adrenal gland. In both cases clinical convalescence occurred. Interest of the presented observation consists in its exclusive rarity. The unilateral adrenalectomy was efficient, but the probability of a recurrence of a hypercorticoidism and other manifestations of Carney complex remains.