We compared the change over time of individual quality of life (QOL) versus health-related QOL (HRQOL) and functional status in palliative care patients with amyotrophic lateral sclerosis (ALS). Forty-two patients with ALS performed the following assessments: the ALS functional rating scale (ALSFRS), the Sickness Impact Profile (SIP), the Short Form 36 (SF-36), and the Schedule for the Evaluation of Individual QOL-Direct Weighting (SEIQOL-DW). The SF-36 and the SEIQOL-DW were assigned at random. Patients were examined at least three times at 2-month intervals. There was a significant decrease from visit 1 to 3 (4-month period) in the function-based values of the SIP, SF-36, and ALSFRS. Despite this progressive decline of physical function and HRQOL, individual QOL as assessed by the SEIQOL-DW remained stable throughout the observation period. Correspondingly, there was a clear correlation between the ALSFRS, the SIP, and the SF-36, but no correlation between these scales and the SEIQOL-DW. The QOL domains most often named in the SEIQOL-DW were family, friends/social life, health, and profession. Thus, individual QOL appears to be largely independent from physical function in severely ill patients with ALS. Because of the characteristics of the scale, assessment of individual QOL may have an interventional value in palliative care.