3 unrelated patients with unexpected or non-syndromic phenotypes, accompanied by a VHL component tumor received germline gene panel testing as part of the PROACTIVE study at the Dana-Farber Cancer Institute, each detected with a low penetrant variant. At age 32, patient 3 (female) presented with posterior headaches, which a CT scan and MRI revealed to be a cerebellar hemangioblastoma. Treatment involved a gross total resection of the lesion. Somatic testing of a left cerebellar mass revealed a somatic VHL variant: p.Leu63fs*4 (c.188_188T>TG); updated to p.R64fs (c.189dupG). Single gene testing of VHL revealed a heterozygous germline VHL p.Pro81Ser (c.241C>T) variant. To this day at age 40, she continues to be monitored. Her mother (age 72) and two of her three siblings (ages 38 and 36) carry the same VHL p.Pro81Ser variant, but have been negative for hemangioblastoma or any other VHL component tumor. ACMG: PP4