Introduction High rates of psychopathology and worse performance in cognitive areas have been described in high risk (HR) first degree relatives of subjects with schizophrenia. The present study aimed to examine clinical and neuropsychological characteristics in two different groups of first degree relatives of patients with schizophrenia – one of siblings (HRs), and one of offspring (HRo) – and compare them with healthy controls (HC). Methods Participants were 30 HRo, 26 HRs and 33 HC, all aged between 7 and 16years. Clinical and neuropsychological assessments were completed by all participants. Results No significant differences were observed between HR groups in DSM-IV diagnoses. The most prevalent diagnosis in both HR samples was attention deficit/hyperactivity disorder (ADHD) followed by oppositional defiant disorder and generalized anxiety disorder. Both HR groups obtained worse scores than HC on prodromal symptoms, premorbid adjustment and behavioral problem scales. In cognitive areas, HRo performed worse than HC on most WISC-IV index scores, logical memory, visual memory and perceptual organization, whereas HRs only performed worse in WISC-IV indexes, logical memory and perceptual organization. Most of these differences remained stable after controlling for ADHD. The comparison between HRo and HRs showed significant differences in prodromal symptoms and working memory after controlling for ADHD. Conclusion Similar abnormalities in HRo and HRs were found in relation to clinical and neuropsychological variables. Subtle differences were found between HR groups with HRo showing difficulties in more clinical and neuropsychological areas than HRs and HC. This suggests that, the specific kind of family relationship should to be taken into account in future HR research.