Spinal muscular atrophy (SMA) is a genetic disorder characterized by degeneration of the anterior horn cells leading to muscle atrophy and weakness. SMA is an autosomal recessive disorder in 95% of the cases. It is caused by homozygous deletion or mutation in the 5q13 survival of motor neuron gene. The severity of spinal muscular atrophy is highly variable, and the clinical features can be classified into four main types based on the age of onset. There is a great excitement in the field since some therapeutic agents have been recently approved for the treatment of spinal muscular atrophy. However, their long-term effect on muscle weakness is not completely known. We present a case of the most severe prenatal type 0 of spinal muscular atrophy. The newborn was a homozygotic carrier of the mutation and survived only for 2 months after delivery.