Histories have been presented of two pairs of sibling children with recurrent macrocytic anemia with megaloblastic bone marrow responding specifically to liver extract and vitamin B12. The clinical features and laboratory findings were similar to those of adult pernicious anemia in every respect except for the inconstant presence of histamine refractory achlorhydria. All the patients responded to vitamin B12 given parenterally, but in three to whom it was given by mouth no response was obtained. Two of these subsequently responded to oral B12 when it was accompanied by normal gastric juice. The other two developed histamine refractory achlorhydria while in hematologic remission. This is interpreted as proof that the anemia was due to deficient intrinsic factor in the gastric juice. Three patients showed evidence of disease of the spinal cord, in two of whom the symptoms were severe. These symptoms were compatible with a diagnosis of dorsolateral sclerosis, and improved with antipernicious anemia therapy. In one of these patients, treated with folic acid, the neurologic lesions were aggravated severely. Pernicious anemia due to the absence of gastric juice intrinsic factor occurs in children. The presence of free hydrochloric acid in the gastric contents does not preclude the possibility of the disease.