Lentigines, atrial myxoma, and blue nevi (LAMB) syndrome/Carney syndrome is a very rare, autosomal dominant, and hereditary syndrome. Seventy percent of individuals with LAMB syndrome have germline inactivating or deleting mutations of the LAMB SYNDROME1 gene [currently known as protein kinase cyclic adenosine monophosphate (cAMP)-dependent type I regulatory subunit α (PRKAR1A), located at the 17q22-24 chromosome level], a member of the lentiginosis family. Dermatological features include skin pigmentation and cutaneous/mucosal myxomas, usually diagnosed by the age of 20 years (neonatal presentation is exceptional, requiring a meticulous differential diagnosis). Melanocyte-derived tumors such as epithelioid blue nevi (with different levels of pigmentation) and pigmented epithelioid melanocytoma (previously “animal-type melanoma”) are often found. Myxomas, mesenchymal tumors with mostly a benign pattern, may be recurrent. Primary cutaneous melanotic schwannoma is atypical, while nonskin sites are frequent. Corticotropinomas or somatotropinomas are part of the hereditary syndrome-related pituitary adenomas (representing 5% of all). The primary pigmented nodular adrenocortical disease involves bilateral cortical hyperplasia causing Cushing's syndrome (CS) at an earlier age than non-LAMB syndrome cases; osteoporotic fractures seem more prevalent compared to other etiologies. Typically benign, a few cases of adrenocortical carcinoma have been identified. A total of 5% of familial nonmedullary thyroid cancer are syndromic, also including LAMB. Lentigines, atrial myxoma, and blue nevi syndrome-related thyroid frame include hyperthyroidism, follicular hyperplasia/adenomas, and follicular carcinoma (usually aggressive, bilateral, or multifocal). Large-cell calcifying Sertoli cell tumors (LCCSCTs) of the testes have malignant behavior in adults; in children, these may induce precocious puberty. Two particular mammary tumors are found: myxoid fibroadenomas and breast myxomatosis. Cutaneous/subcutaneous lesions, pigmented or not, or any focal swelling of nonidentified cause needs careful examination since dermatological elements are among the earliest and most discernible by which to detect lesions in LAMB syndrome, a systemic condition with multilevel endocrine involvement.