Introduction: Spinal Muscular Atrophy (SMA) is one of the most common neuromuscular diseases. In recent years the natural history of this disease has been found to change.

Methods: To describe SMA brazilian children  we adopted the data base information of a global registry. Information input, regarding demografic, motor function , pulmonary, orthopedic complications and molecular tests comes from medical documentation.

Results: This is a ongoing project in line with global registries. At present only the parcial results are described. A total of 46 patients have had their information colected. Ten were classified as SMA type 1, with an age range from 1 to 13 years, 6 boys, feeding by gastrostomy, need for ventilatory support from 11 to 24 hours daily. Only two have died. SMA type 2 is the largest group with 20 pacients, from 2 to 23 years of age, twelve males, either sitting with support or standing with support, scoliosis (36%) and respiratory support (27%) were the main complication in this group. Sixteen type 3 SMA, from age 5 to fifty one, 9 females, either standing or walking, some were overweight and only one had scoliosis and hypoventilation. SMN2 copy number were higher in type 3 (4 or 5 copies), than in type 2 (2 or 3) and type 1 (0 to 2).

Conclusion: As in other countries SMA has nowadays a longer span of life also in Brazil.