Lipomatous tumours are rare in the head and neck region. Their biological behaviour varies greatly, from absolutely benign to histologically benign but locally infiltrative and, finally, invasive with metastatic potential. Each lipomatous tumour has to be treated accordingly. It is of paramount importance before eventual surgery is planned to perform adequate imaging, diagnostic biopsy and careful assessment. Only in small (<5 cm), superficial soft tissue tumours or when magnetic resonance imaging has demonstrated specific features of lipoma, may diagnostic biopsy be omitted. In these cases, expectant management or simple excision is appropriate. Adequate preoperative diagnosis is important to assure adequate tumour control as well as optimal functional and cosmetic outcome. The major problem in the treatment of lipomatous tumours of the head and neck region is the presence of nearby delicate structures. Especially, wide surgical excision of liposarcomas may be hindered by anatomic constraints and may result in impaired functional and cosmetic outcome. Neoadjuvant radiotherapy and specific systemic chemotherapy may be helpful in the treatment of liposarcoma, especially when unresectable or when primary surgery is expected to result in poor oncological, functional or cosmetic outcome. Greater emphasis placed on the underlying biology of individual sarcoma subtypes, development and evaluation of novel therapies and greater specificity in the selection of chemotherapy agents based on activity in individual histological subtypes are expected to lead to improved efficacy of systemic treatment.