Here we report a very peculiar ALS case bearing both a novel missense mutation within the spastin gene (SPG4), the commonest causative gene for HSP, and at the same time the homozygous deletion of the SMN2 gene, the centromeric copy of the SMA defective gene (SMN1), known to have the potential to mitigate the clinical phenotype in SMA [7, 8] and, possibly, in ALS [9, 10], albeit in this latter case with conflicting results [11]. This evidence concerns the gene SPAST and hereditary spastic paraplegia.