Spitzoid morphology involving at least 25% of the tumor was observed by at least 3 dermatopathologists in 77% (23 of 30), 75% (3 of 4), 50% (2 of 4), and 50% (1 of 2) of melanomas from individuals with germline variants in POT1, TERF2IP, ACD, and TERT, respectively. The gene discussed is ACD; the disease is neoplasm.