ObjectiveThe objective of the present study is to demonstrate the potential of dedicated neurosonography for the diagnosis of fetal brain involvement in tuberous sclerosis complex.MethodsIn this multicenter retrospective study of fetuses at high risk for tuberous sclerosis complex we review dedicated neurosonographic, fetal MRI and postnatal reports. The data reviewed included reason of referral, gestational age in which the cardiac rhabdomyomas were first suspected and final number of cardiac rhabdomyomas detected in the dedicated scan. We search for tuberous sclerosis complex ‐related brain involvement looking for the presence of one or more of the following findings: a) white matter lesions; b) subependymal nodules; c) cortical/subcortical tubers and d) subependymal giant astrocytoma.ResultsWe found 20 patients at risk, 19 because of the presence of cardiac rhabdomyomas and one because a deletion in chromosome 16 involving the tuberous sclerosis complex gene site. Cardiac rhabdomyomas were diagnosed at a mean gestational age of 27w2d (range 16w0d‐36w3d), the mean number of cardiac rhabdomyomas was four (range 1‐10). Brain involvement was present in 15 fetuses, in 13 cases the disease was confirmed by one or more ways: chromosomal microarray (1), exome sequencing (7), autopsy findings (4), and newborns with clinical tuberous sclerosis complex (4), sibling diagnosed with clinical tuberous sclerosis complex (1). In two cases the disease couldn't be confirmed: lost to follow up (1), autopsy not performed (1). In five cases without brain findings, exome sequencing (2) or autopsy findings (1) confirmed tuberous sclerosis complex; in the two remaining cases, exome sequencing was normal but in one of them they were five cardiac rhabdomyomas and the last case the autopsy was considered normal, representing the only false positive case.ConclusionsContrary to current literature, dedicated neurosonography appears to be effective in the diagnosis of tuberous sclerosis complex brain involvement in fetuses at risk and should be use as the first line approach. Although the number of cases where MRI was performed was small is seems that mainly in the presence of US findings the added value of MRI is low.This article is protected by copyright. All rights reserved.