The Mayer-Rokitansky-Kuster-Hauser (MRKH) is a syndrome of unknown etiology characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in women showing normal development of secondary sexual characteristics. We report the case of a patient with vaginal aplasia and schizophrenia presenting with sexual delusion. To the authors’ knowledge this is the first case to provide evidence of coexistence between MRKH and sexual delusion in a schizophrenic patient. The core of the patient’s delirium was that she was having sexual intercourse with an eminent person through the big toe of her right foot. We approached this case using a neurological and a psychodynamic hypothesis. The neurological hypothesis suggests that the “deactivation” of the patient’s genitalia led to an expansion of the adjacent big toe cortical area. The psychodynamic hypothesis supports that the sexual function and pleasure was partially expelled from the body image and was stored in a non sexual part of the body (i.e., big toe). Clinicians should be aware of this association and offer patients with MRKH psychological or/and psychiatric evaluation.