For the first time, we describe the autopsy of a patient with SCA34, with a known c.736T>G, p.W246G (NM_022726.4) mutation in ELOVL4. Neuropathological findings include marked neuronal loss and pontocerebellar fiber degeneration in the pontine base, CD68-positive macrophages laden with periodic acid-Schiff (PAS) and Gallyas-positive material, and oligodendrocyte and myelin degeneration with frequent cerebral white matter vacuoles. Here, ELOVL4 is linked to spinocerebellar ataxia type 34.