Background: Maffucci´s syndrome is a rare genetic disorder that affects the skeletal and venous systems. It is also known as dyschondrodysplasia with hemangiomas, enchondromatosis with multiple cavernous hemangiomas, Kast syndrome, hemangiomatosis chondrodystrophica, and enchondromatosis Spranger type II. There have been only reported less than 200 cases in the world. Case Report: This case presents a 25-year-old male with malformations-deformations of the skeletal and venous systems: upper limbs with limbs asymmetry, shortening of the ulna with radius arching. Lower limbs examination showed, varus or valgus angulation of the knee and deformities of the ankle, adequate sensitivity, strength, trophic, without paresis, paresthesias, myalgias, normal preserved ROTS in 4 limbs. Conclusion: To diagnose Maffucci´s syndrome it is essential to perform a thorough medical history, clinical and radiological examination. There are genetics analysis that help the diagnosis but are still under study. Management entails a very strict follow-up and careful examination and monitoring for malignant degenerations.