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Despite advances in rheumatological treatments, patients with systemic sclerosis (SSc) suffer with myriad symptoms and experience associated quality of life decrements. Although SSc-related mortality is reported around 53%, there is a paucity of existing literature describing advance care planning and palliative care in SSc. Here we review symptom management burden and strategies by organ manifestation: interstitial lung disease, pulmonary hypertension, gastrointestinal involvement, cutaneous and musculoskeletal manifestations and digital ischaemia. Then we review domains of palliative care in the context of SSc including psychosocial support, sharing prognostic information, discussing goals of care, advance care planning and comfort-focused care. Finally we suggest unmet research needs in palliative care and SSc.