The invention discloses Chinese and Western compound medicine for treating progressive spinal muscular atrophy and a preparing method thereof, and belongs to the technical field of medicine. The medicine is prepared from large-leaved chrysosplenium, sweet pomegranate, semen coicis, pine needles, herba epimedii, ficus lyrata, heterophyllous wing seedtreeroot, nude fern herb, silverweed cinquefoil root, David's deerhorn, cajeput, radix achyranthis bidentatae, radix asparagi, tarenna attenuata, elemental diet, ambenonium chloride, piribedil and neostigmine. The adopted Chinese and Western medicine combined treatment method has a better effect than that of just using traditional Chinese medicine or Western medicine, with the treatment rule of nourishing liver and kidney, strengthening the muscles and bones, dispelling wind and removing obstruction in the meridians, nourishing heart, tonifying qi and moistening and promoting granulation, the traditional Chinese medicine components and Western medicine effects are improved with the maximum efficiency, complementation and promotion functions are brought into play, the medicinal effect is improved, central nervous systems of nerves, internal secretion and immunity of the organism are stimulated to carry out stress response, and the Chinese and Western compound medicine has good treat effects on muscular atrophy, skin numb, myasthenia, periodic paralysis and myodystrophy caused by progressive spinal muscular atrophy.