BACKGROUND In lymphedema, an imbalance in the formation and absorption of lymph causes accumulation of protein-rich fluid in the interstitium of the most gravity-dependent parts of the body. Diagnosis is usually made based on patient medical history and a physical examination showing a typical appearance of the affected body part. Differential diagnosis is confirmed by imaging. CASE REPORT Primary lymphedema is inherited in through an autosomal dominant pattern. Congestive cardiac failure and non-filarial infections predispose patients to the secondary form of lymphedema, elephantiasis nostras verrucosa (ENV). We present the case of a 65-year-old man with lymphedema praecox complicated by congestive cardiac failure. The patient was experiencing worsening left leg swelling and had a prior history of unilateral leg swelling at puberty. The condition was inherited through an autosomal dominant pattern, as his father, elder brother, and nephew were diagnosed with the same disease. The left leg showed non-pitting edema with indurated, woody skin and lichenification. The right leg had mild pitting edema. There were numerous verrucous folds and cobblestone-like nodules, and plaques and a painless ulcer on the left leg. Laboratory evaluation demonstrated an elevated B-type natriuretic peptide. He was treated with compression stockings and inelastic multi-layer bandaging and was administered limb decongestive treatment. After 1 week of therapy, his swelling had somewhat improved. CONCLUSIONS Various conditions can cause ENV and it can superimpose on any form of hereditary lymphedema. The most effective strategy for this condition seems to be a thorough workup of the underlying cause of the ENV and early intervention.