In April 2007, two newborn springboks (Antidorcas marsupialis) from a zoo of southern France were found dead. Necropsy was performed on the two animals and revealed arthrogryposis, mild facial structural abnormalities, and bilateral enlargement of the kidneys with concurrent hydronephrosis in both newborns. Histopathologically, extensive cytoplasmic vacuolation of neurons in the central nervous system, thyroid follicular epithelium, renal tubular epithelium, enterocytes, hepatocytes, and ruminal epithelial cells was the most remarkable lesion in both animals. By electron microscopy, the vacuoles were membrane bound and contained scattered membranous and granular material within a primarily electron-lucent background. Hence, a diagnosis of lysosomal storage disease was established, with gross, histological, and ultrastructural features suggestive of an inherited form of mannosidosis. This report documents the first case of lysosomal storage disease in springboks.