Lysosomal storage disease, metabolic diseases and the neuraxonal dystrophies are a highly diverse and heterogenous group of disorders, many of which are defined genetically, but the fetal pathology of these devastating diseases is poorly documented. Here we briefly review the fetal pathology of the lysosomal storage disorders associated with Gaucher disease, ATPV1 and BORCS5 related disease, Type IV Glycogenosis, Neu-Laxova syndrome, glutamine synthetase and adenylosuccinate lyase deficiencies, Zellweger spectrum disorders 3-b-hydroxysteroid-D8, D7-isomerase deficiency and mitochondrial cytopathies.