Situs inversus totalis is a very rare anomaly. Even more, cases of situs inversus totalis plus hypertrophic osteoarthropathy. A woman presented with cyanosis, clubbing of fingers and distal edema. Chest examination revealed heart sounds at the right side and a heart murmur. Abdominal examination showed liver dullness in the left side. The X-rays showed dextrocardia and liver in the left side. The Upper digestive barium test showed the stomach in the right side, the barium enema showed caecum on the left. Treatment for heart failure improved the patient's condition and she remained well. When she became pregnant, developed heart failure again and required admission. She was compensated and continued her pregnancy. 3 years later she developed heart and respiratory refractory failure, was admitted again and after few days she died. We report this case of situs inversus totalis in association with hypertrophic osteoarthropathy because of the rarity of this anomaly.