The treatment of soft tissue and bone sarcoma of the hand and wrist are based on general principles rather than on specific data due to the rare incidence of cases. The current study was conducted to analyze the survival and functional outcomes of patients with sarcoma of the distal part of the upper extremity. Outcome of 168 consecutive patients with soft tissue and bone sarcoma of the hand and wrist were treated over a 34 year period by a single surgeon were retrospectively reviewed. The survivorship was determined using the Kaplan Meyer method. Functional outcomes were evaluated using the Michigan Hand Questionnaire. The overall survival demonstrates the statistical significance (p = 0,002) between patients with low (94,7% (95% CI, 92,9–96,5)) and high (72,7% (95% CI, 71,9–73,5)) grade tumors. There is no statistical significance in overall survival among different subgroups of high-grade sarcomas. Despite the predominant number of patients after external R1/R2 resection, local recurrences and a high number of high-grade sarcoma, the incidence of regional relapse was 12,5% using multimodal approach. The overall hand function reached 72,3 points for the treated hand compared to 92,3 for uninvolved hand (p < 0,001). The series describes the treatment of consecutive cases since 1983 using limb-preserving therapy in single site location. The patients suffering from sarcoma of the hand and wrist should be treated in high volume centers with a sufficient diagnostic and therapeutic infrastructure, including specialized hand and reconstructive plastic surgical specialists.