Turner syndrome (TS) is a neurogenetic disorder characterized by partial or complete monosomy-X, usually resulting of a sporadic chromosomal nondisjunction. It is one of the most common sex chromosome abnormalities, affecting approximately 1 in 2,000 live born females. There are sporadic few case reports of concomitant TS with schizophrenia worldwide. No defined psychiatric condition has been traditionally related to TS, and it is not mentioned in DSM-IV. Although it is not associated with any psychiatric syndrome, several case reports in the literature describe a similar constellation of symptoms in TS that may represent a biologically-based entity. Aripiprazole once-monthly is a second generation antipsychotic recently developed. Its efficacy and non-inferiority to oral aripiprazole have been demonstrated in preventing relapse in patients with schizophrenia. Experience with oral aripiprazole and the current availability of the long-acting formulation suggest a potential benefit in a variety of clinical scenarios and therefore consideration as a treatment option in the treatment of schizophrenia and psychotic symptoms in several disease like TS.