A 62-year-old Caucasian man presented with a 13-month history of a growing, large mass on his upper back...the patient also had 11 genomic variants of known significance including PTCH1 (splice site 1504-1G>T), ASXL1 Q760, INPP4B W521, KEL R130Q, PIK3R1 R534, PTEN (splice site 210 2A>T), RAC1 P29S, TERT promoter-124C>T, TP53 R196, TP53 Q100, and WT1 C350R...he was started on the oral Hedgehog inhibitor vismodegib 150 mg daily with nivolumab...He attained a CR after 5 months of treatment...Follow-up imaging with PET/CT scan remained negative for metastatic disease. Currently, he has been off therapy for 12 months with ongoing CR 20 months from the time treatment was initiated.