Background Aggressive angiomyxoma (AAM) is a locally infiltrative mesenchymal tumour that most commonly affects the pelvis and/or perineum in adult women. AAM is very rare in males, especially in infancy. Case presentation A 10-month-old fulani (African) male infant was referred to our department for a large painless mass in the right testicule. The mass was detected during the neonatal period and gradually increased in size. Ultrasound examination revealed a large heterogeneous lesion; computed tomography results led to the conclusion that the mass was a mesenteric hernia. An inguinal and scrotal surgical approach was adopted. Exploratory surgery found a normal right testicle displaced upwardly and a large scrotal mass. Radical excision of the mass and orchidopexy were performed. Subsequent histology and immunohistochemstry studies indicated that the mass was a scrotal angiomyxoma. The postoperative course was uneventful. No recurrence occurred during the 6-month follow-up. Conclusion To the best of our knowledge, this is the youngest patient with AAM reported to date. Angiomyxoma should be included in the differential diagnosis of scrotal masses, for which radical excision is justifiable to prevent recurrence.