Cutaneous myxoma(s) occurred in 22 (54%) of 41 patients with the complex of myxomas, spotty pigmentation, and endocrine overactivity. Of the 16 patients who had cardiac myxoma(s), the cutaneous tumor(s) was (were) detected in 13 (81%) of them prior to diagnosis of the cardiac neoplasm. Thus, the cutaneous tumor may herald a potentially fatal cardiac neoplasm (and other important conditions as well). Clinical features of the lesion were as follows: early appearance (mean age, 18 years); multicentricity (71% of patients); small size (usually less than 1 cm in diameter); widespread distribution but with predilection for certain sites (eyelids, ears, nipples); and tendency for recurrence. Pathologic features included the following: location in dermis, subcutis, or both; sharp circumscription (sometimes encapsulation); hypocellularity; abundant myxoid stroma; prominent capillaries; lobulation (larger lesions); and occasional presence of an epithelial component.