The aim of the present study was to report a case of congenital upper limb hypertrophy associated with lymphedema in a child. An eight-year-old girl presented with a history of right upper limb oedema since birth, which was more significant in the hand. Volumetry was performed, revealing 1,838 grams in the right arm and 1,483 grams in the left arm. Intensive treatment with the Godoy method® was proposed. Treatment involved five hours per day using the RAGodoy® device, 15 minutes of cervical lymphatic therapy, and three hours of manual lymphatic therapy per day. With treatment, the volume of the right arm was reduced to 1,663 grams (reduction of 205 grams). After treatment, hypertrophy of the limb was noticed, which had been aggravated by the lymphedema. Congenital hypertrophy of the upper limb is rare in children. Its association with lymphedema has not previously been reported in the literature and could cause diagnostic difficulties.