This review presents 21 cases, found in the literature, of a CNS lesion (a tumor in 19 of them) associated with emaciation, anorexia and several psychic symptoms that had led to the diagnosis of anorexia nervosa (AN). Anorexia and psychic disturbances preceded the neurologic signs and/or the correct diagnosis in all patients (by a mean of 2.9 years, range = 0.2-17 years). Anorexia had begun before the age of 25 years in 18 patients of which two-thirds were females. Only a few cases fulfilled the DSM-III-R criteria for AN; the majority could be characterized as 'atypical AN'. Although AN is usually conceived as a primarily psychogenic disorder, structural lesions of the hypothalamus (or other sites involved in food regulation) in animal models and in these human cases mimic many features of AN, suggesting the possibility of an as yet unidentified structural hypothalamic disorder to be implicated in the etiopathogeny of AN. The unusually high incidence of germ-cell tumors in this review (33%) suggests that they are more likely than other tumors to influence the limbic system toward an anorectic syndrome.