Enlarged mitochondria are observed in a number of sensory neuropathies (see Vital & Vital, 2012; for review) and it is of interest that dominant mutations in SPTLC1 (Bejaoui et al., 2001; Dawkins et al., 2001) and SPTLC2 (Penno et al., 2010; Oswald et al., 2015) that generate aberrant sphingolipids give rise to Hereditary and Sensory Neuropathy Type 1 (HSAN1) where enlarged mitochondria are often observed. The gene discussed is SPTLC2; the disease is hereditary sensory and autonomic neuropathy type 1.