Background: The association of complete androgen insensitivity syndrome (CAIS) and malignancy has only been described in case reports worldwide. As such, risk association of tumorigenesis is considered lower and related to age, in comparison to partial androgen insensitivity syndrome (PAIS). Case Presentation: We report on a 24-year-old patient presenting with palpable hypogastric mass with concomitant primary amenorrhea. Work-up for CAIS was done with the pelviabdominal mass revealing a seminoma by histopathology. Surgery and adjuvant chemotherapy using BEP protocol (Bleomycin- Cisplatin- Etoposide) was offered successfully showing absence of tumor recurrence on end of treatment evaluation. Conclusion: The complication of malignancy pose challenge to clinicians for early diagnosis and treatment with androgen insensitivity syndrome. More so, a multidisciplinary team that will involve the patient and family in making informed decisions on issues of available treatment options, gender assignment, and counselling is crucial in order to promote well-being and to aim at minimizing physical and psychosocial risks.