Priaprism is a persistent painful erection that continuous beyond or is unrelated to sexual stimulation. Majority of cases are idiopathic (46%), alcohol and drug related (21%), perineal trauma (12%), sickle cell anemia and hypercoagualable state related (11%). We report case of priapism caused by hyperhomocysteinemia with favorable outcome with only few cases so far reported in the literature to the best of our knowledge. A 31 year-old male referred to our institution with non resolving priaprism for the last 6 days. Immediate distal shunt (Al-ghorab) was created but it could not achieve the detumescence. The penile Doppler showed no flow into the corpora, so a proximal shunt (Quackels) was made which achieved satisfactory detumescence. On thorough evaluation for the cause of priaprism, only homocysteine level was found to be significantly raised (40.46 µmol/L), being the unusual and rare cause for priaprism. The patient was discharged on homocheck. In the follow-up the patient is on vacuum assisted device for the erectile dysfunction and has been advised for the penile implant. Priaprism being a urological emergency should be thoroughly evaluated even for the rare causes and should be timely intervened to avoid the unavoidable consequences of permanent erectile dysfunction.