Clear cell sarcoma (CCS) is a rare soft-tissue sarcoma and is characterized by a translocation t(12;22) (q13;q12). Because of some similarities to maligned melanoma it is called melanoma of soft parts. CCS has characteristic clinical features. It is commonly reported in young adults as slowly growing tumor followed by aggressive course. Its features are: regional lymph node spread and tendency for local recurrence and a propensity for pulmonary metastasis. Metastatic CCS is a very aggressive disease with pure prognosis. Conventional chemotherapy has little role to play in the management of CCS. The article presents the case of a patient with metastatic CCS with massive lung dissemination, metastatic in liver, left adrenal, pelvis and superficial soft tissue. In patient pazopanib therapy resulted in nine-month control of the disease.
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