Frontotemporal dementia (FTD) is a progressive neurological condition caused by degeneration of the frontal and/or anterior temporal lobes resulting in personality, behavioral, and cognitive changes. Amyotrophic lateral sclerosis (ALS) is caused by degeneration of lower motor and pyramidal neurons, leading to loss of voluntary muscle movement. The common molecular pathological and anatomical overlaps between FTD and ALS suggest that the two disorders are strongly linked. In some patients FTD precedes ALS; in others ALS occurs first, while in still others the two disorders begin simultaneously. The association between ALS and FTD creates unique challenges for family caregivers. This paper provides a guide for healthcare providers caring for patients with FTD-ALS exhibiting behavioral, cognitive, and emotional symptoms. Strategies are suggested to help minimize the impact of negative symptoms.