The main clinical manifestations of this case were spastic paraplegia with darkening of the skin and hyperpigmentation of the lips, intraoral mucosa, and areola starting during adolescence; decreased plasma cortisol levels and increased adrenocorticotropic hormone (ACTH) levels; subclinical peripheral nerve damage found in nerve conduction velocity; high serum VLCFAs detected; genetic testing showed a missense mutation in exon 1 of the ABCD1 gene with c.761C>T. Here, POMC is linked to Spastic paraplegia.