In this paper, we report a case of a 23-year-old male patient with bilateral absence of the flexor pollicis brevis and abductor pollicis brevis muscles with an intact functioning opponens pollicis and flexor pollicis longus muscles with bilateral thenar atrophy due to its rarity. All physical, neurological, ultrasonographic, direct radiographic, electromyographic and MRI studies were used to confirm and document this congenital anomaly.