Background: Wilms’ tumor (nephroblastoma) is the most common solid primary renal malignancy in children but extremely rare in adult population. We report the clinical presentation and therapeutic results of our adult Wilms’ tumor patients and review the previous literatures about diagnosis and treatments. Methods: A retrospective review was carried out to investigate the patients with adult Wilms’ tumor at the Tri‑Service General Hospital during a 35‑year period (1980–2014). Seven patients ≥16 years old (one female and six males; median age at the time of diagnosis of 24 years; range: 21–48 years) were staged according to the National Wilms’ Tumor Study staging system as follows: three patients had Stage II disease, two patients had Stage III disease, and two patients had Stage IV disease. All statistical values were represented as mean ± standard deviation. All tests were two‑sided, with P < 0.05 considered statistically significant. All statistical analyses were carried out using SPSS software (IBM, Armonk, NY, USA). Results: The most common clinical presentation is flank pain (57%) followed by palpable mass (43%). Only one patient presented with gross hematuria. Four patients underwent renal arteriography, which all disclosed irregular, tortuous vessel pattern with faint tumor stain. All patients underwent radical nephrectomy (one – hand‑assisted laparoscopic nephrectomy and six – open transperitoneal nephrectomy). Two patients developed lung metastases after radical surgery. Six patients received multimodal therapy, including surgery, chemotherapy, and radiation therapy. The mean disease‑free survival is 81 months. The 3‑ and 5‑year survival rates are 83% and 71% separately. Conclusions: Selective renal arteriography could provide a valuable diagnostic hint in adult Wilms’ tumor. Using multimodality therapy, improved prognosis could be achieved.