Here, we characterized clinical, histological and genetic correlates of familial and sporadic patients with TRPC6-AP.<h4>Methods</h4>In this multicentre observational study, an online questionnaire followed by a systematic literature review was performed to create a cohort with comprehensive data on genetic and clinical outcomes [age of onset, clinical presentation, treatment response, kidney biopsy findings and progression to kidney failure (KF)]. Here, TRPC6 is linked to kidney failure.