Situs viscerum inversus totalis is a rare disorder presenting with complete transposition of thoracic and abdominal viscera. It is associated with certain organ anomalies, but it is not a predisposing factor to cholecystolithiasis. To date, fewer than 100 cholecystectomies in patients with situs inversus have been published worldwide. We report a case of a 75-year-old woman referred to our hospital with abdominal pain in the left hypochondrium. Situs inversus totalis and acute calculous cholecystitis were diagnosed and successfully conservatively treated with antibiotics. The patient underwent elective laparoscopic cholecystectomy 3 months later. The procedure and postoperative course were uneventful and the patient recovered well. The clinical presentation of these patients with cholecystolithiasis may be confusing and vague and the correct diagnosis delayed. Laparoscopic cholecystectomy is the gold standard in the treatment even though the operation requires some modifications in operating theatre arrangement and position of the surgical team. Most surgeons are right-handed, and to operate laparoscopically in the "mirror image" anatomical situation using mainly the left hand for dissection may be stressful, uncomfortable and more time-consuming. Some recommendations to overcome this issue have been published. In conclusion, the above-mentioned anomaly may cause some risk and delay of the exact diagnosis, but it is not dangerous in itself. Laparoscopic cholecystectomy is a safe procedure, even in the case of acute cholecystitis, if performed by an experienced laparoscopic surgeon. The most dangerous is always an incautious and too self-confident surgeon.