Introduction: Angioleiomyomas are rare benign tumors originating from smooth muscle cells of blood vessels. Although they can occur in various anatomical locations, angioleiomyomas of the distal leg are relatively uncommon. Due to its clinical resemblance to other soft-tissue tumors, misdiagnosis can occur leading to inadequate treatment. Case Report: We present a case of angioleiomyoma in a 54-year-old female who presented with a palpable mass in her distal leg. The tumor was surgically excised, and histopathological examination confirmed the diagnosis of angioleiomyoma. In this article, we discuss the clinical presentation, diagnostic evaluation, and management of angioleiomyoma, with a focus on distal leg tumors. Furthermore, we provide a comprehensive review of the existing literature on angioleiomyomas, emphasizing findings and treatment outcomes reported in previous studies. Conclusion: Angioleiomyomas are uncommon soft-tissue tumors that can mimic other more common lesions such as ganglion cysts. Hence, diagnosis requires a high index of suspicion. Surgical excision is the treatment of choice for angioleiomyoma. Complete resection is generally curative, with a low rate of recurrence. Keywords: Angioleiomyoma, distal leg, surgical excision, review of literature.