A 37-year-old man with no symptoms or family history of tuberous sclerosis complex presented to our hospital with abdominal pain in 2013. Abdominal computed tomography (CT) revealed a rupture in the right renal angiomyolipoma (AML) having a maximum diameter of 7 cm. He had undergone a transarterial embolization. Follow-up CT showed an increase in the size of the right tumor to 11 cm, and therefore, right nephrectomy was performed in 2016. The diagnosis of epithelioid AML (EAML) was confirmed. In 2019, he was diagnosed with a solitary tumor near right-sided transverse colon, which was resected and showed recurrence of EAML. He was disease-free 6 months after surgery. EAML has malignant potential, with 30-50% of reported EAML cases resulting in local recurrence or distant metastasis. Previous recurrence or metastasis may occur 0.25-12 years postoperatively. Furthermore, multiple and unresectable recurrences or metastases, arising early in the postoperative period may lead to a poor outcome. Therefore, close and long-term follow-up is required.