This workshop, organized by Michael Benatar and Terry Heiman-Patterson, will bring together diverse stakeholders across the ALS and FTD community, including clinicians, scientists, genetic counselors, ethicists, and members of the at risk genetic ALS/FTD community, to develop guidance for the clinical management of individuals at significantly elevated genetic risk for ALS and FTD. With increasing knowledge of the genetic causes of ALS and the more routine use of clinical genetic testing in the ALS patient population, we are seeing an increasing number of family members seek pre-symptomatic testing. Moreover, many of those found to carry a genetic mutation are seeking clinical care, with questions about off-label use of FDA approved medications and lifestyle modifications. In the absence of good evidence, it is critical to develop guidance for clinicians who are tasked with providing care for these individuals at significantly elevated genetic risk for ALS and FTD. The goal of this workshop is to begin to develop these guidelines and identify the gaps in knowledge.