Objective: Osteogenesis imperfecta (OI) is regarded as a heterogeneous clinical condition. Genetic complexity and extensive phenotypic variation lead to heterogeneous clinical manifestations in the condition. Methods: A 33-year old, male underwent consultation because of irritability, reference ideas and aggressive behavior. Results: OI type-IV was diagnosed on the basis of physical examinations and radiological findings. Psychotic symptoms improved following low-dose antipsychotic treatment. Discussion: This report presents a rare case of OI with psychotic symptoms. Comorbidity of a psychiatric disorder and OI in a case with a positive family history for both may also emphasize a common genetic basis between the psychotic disorders and OI.