Phlegmasia cerulea dolens (PCD) is a fulminant condition of acute massive venous thrombosis that may result in major amputation or death unless treated in an early phase. Guidelines for treatment are still not clearly documented. As a consequence, physicians might have limited knowledge of this potential life-threatening condition and its clinical course. Therefore, the purpose of this review was to analyze and summarize clinical manifestations and proposed diagnostic approach, factors that affect the outcome of PCD, and the evolution of management and therapeutic options. Underlying malignancy, pulmonary embolism, and PCD severity are the vital factors that predict the outcome of PCD. In the last decades, treatment options have remained largely unchanged. Published evidence shows that advances in minimally invasive techniques have not yet resulted in outcome improvements compared with traditional surgical thrombectomy. Treatment seems to depend on grading the severity of this condition and experience of the surgeon.