Osteogenesis imperfecta (OI) is regarded as a heterogeneous clinical condition. Genetic complexity and extensive phenotypic variation lead to heterogeneous clinical manifestations in the condition. A 33-year old, male underwent consultation because of irritability, reference ideas and aggressive behavior. OI type-IV was diagnosed on the basis of physical examinations and radiological findings. Psychotic symptoms improved following low-dose antipsychotic treatment. 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. [Anadolu Psikiyatri Derg 2017; 18(0.100): 23-25]