Abstract: Spinal muscular atrophy (SMA) is a rare hereditary neuromuscular disease. Clinical manifestations of SMA include proximal limbs symmetrical and progressive muscle weakness and atrophy. Children with SMA are susceptible to swallowing dysfunction, gastrointestinal dysfunction, feeding difficulties, abnormal bone metabolism, and undernutrition/overnutrition. Currently, there is no standardized SMA nutrition support guideline. Patients need regular evaluations of growth and nutritional status. Nutritional interventions and treatments need to be done by professional dietitians. Individualized nutrition management plays an important role in improving the quality of life, delaying the illness, and prolonging the survival time of children with SMA.