Pernicious anemia is uncommon in Africa. The purpose of this report is to describe a case of pernicious anemia observed in Madagascar. The revealing manifestation was encephalomyelitis with combined medullar sclerosis that responded favorably to vitamin B12 replacement therapy. Clinical symptoms included paresthesia associated with allodynia of all four extremities and with tetrapyramidal syndrome, medullar ataxia and minor cognitive disturbances ongoing for 5 months. Hemogram testing revealed macrocytic anemia. Serum cobalamin level was low. Anti-intrinsic factor antibody was detected. Spinal cord magnetic resonance imaging showed diffuse high-signal intensity along the posterior spinal cord extending from C1 to C4. Vitamin B12 replacement therapy led to full regression of clinical signs after six weeks. Association of central nervous system involvement with macrocytic anemia suggests vitamin B12 deficiency and pernicious anemia should be suspected. This disease can be considered as a curable form of myelitis in Africa and Madagascar.