Acute symptomatic upper extremity deep vein thrombosis (DVT) are estimated to account for only 2-4% of all deep vein thrombosis. Upper extremity DVT leading to phlegmasia cerulea dolens (PCD) occurs in an estimated 2-5% of these cases. Progression of PCD to venous gangrene is extremely rare with only 16 previously reported cases in the literature. Only 7 of the cited cases document significant tissue loss. This report describes a 61-year-old male who developed upper extremity DVT complicated by PCD which led to venous gangrene and limb loss.