Introduction: Hernias containing the appendix are well-known entities that preferentially affect the groin. The presence of an appendix within a Spigelian hernia, which is a rare ventral hernia that presents with nonspecific symptoms and location, adds significantly to the complexity and rarity of its diagnosis. Further, only 18 such cases were reported and published in the literature. We report a case of Spigelian hernia and appendicitis within the hernial sac to highlight the diagnostic challenges associated with this type of hernia. Case Presentation: In her late 30s, a female who underwent laparoscopic surgery for a uterine fibroid two and a half years back presented with right lower quadrant abdominal pain for two days. This followed abdominal pain on exertion for the previous nine months. On examination, there was tenderness, guarding, and a positive cough impulse in the right lower quadrant of the abdomen. Her body mass index was 37.53 kg/m2. A contrast-enhanced computed tomography imaging of the abdomen reported a right iliac fossa incisional hernia with omental congestion necessitating an emergency laparoscopic surgery. At surgery, a Spigelian hernia, which was at least 5 cm away from the port site scar of the previous surgery, was found. The hernia contained an inflamed appendix. Thus, a diagnosis of Spigelian hernia and appendicitis within the hernial sac was made. A laparoscopic reduction and repair of the Spigelian hernia and a laparoscopic appendectomy were performed. Conclusion: Whereas Spigelian hernias are considerably rare, the presence of an appendix within a Spigelian hernia sac is yet a more infrequent occurrence. Although the imaging modalities are often inconclusive, careful interpretation of the computed tomography imaging findings may reveal the pathology preoperatively.