Purpose We analysed 11 patients with malignant musculoskeletal tumours of the humerus who underwent limb salvage surgery with total humeral custom endoprosthesis from 1990 to 2009. Methods There were six male and five female patients, with a mean age of 17 years. The most common diagnosis was osteosarcoma. The average follow-up period was 66 months, with the maximum being 180 months. Functional and oncological outcomes were analysed. Results The one and five year cumulative survival (Kaplan-Meier method) rates were 90.9% and 77.9 %. The average Musculoskeletal Tumour Society Score (MSTS) was 80%. Two patients died due to metastasis. One patient had a forequarter amputation for local recurrence. The procedure provides fast recovery and relatively good restoration of elbow function, whereas active shoulder movements remain limited Conclusion Total humeral custom endoprosthetic replacement represents a viable treatment option in indicated patients, providing reliable and reasonable function of the upper limb, with a low complication rate.