Premature graying of hair (PGH) being a very common entity for which pharmacotherapy and reversibility are not properly addressed. Therefore, this systematic review was conducted to address these issues. For this relevant study were selected from various databases including PubMed, EMBASE, OVID, Web of science, Scopus, and Google Scholar till January 20, 2019. Studies which reported risk factors, co-morbid conditions associated with PGH, its pharmacotherapy and reversal were included in the study. Although many risk factors are reported in literature, smoking, vitamin deficiency (B12, folic acid, and B7), mineral deficiency (low serum calcium and serum ferritin) are found to be associated with PGH. Other important risk factors are family history of PGH, obesity, high B.P, lack of exercise, drugs, genetic syndromes, dyslipidemia, thyroid disorders, hyperuricemia, and alteration in liver function. PGH is found to be an important marker of CAD, more so in case of smoker. Among different pharmacotherapeutic management options, low grade recommendation (2A) is given to calcium pantothenate, PABA, calcium pantothenate + PABA combination. Anu-tailam is the only herbal agent evaluated in clinical research settings. Finally, treating the accompanying pathologies led to the reversal of the disease in many cases. K E Y W O R D S co-morbidities, premature graying of hair, reversal, risk factors, treatment 1 | INTRODUCTION Hair graying is a part of aging, which typically starts around 34.2 ± 9.6 years (whites) to 43.9 ± 10.3 years (in blacks). 1 Premature graying of hair (PGH) is said to occur if hair graying starts before the age of 20 (Caucasians) and 30 years in Africans. 1-4 For Indian people, a cut off range of 25 years is suggested. 1,5 DOPA oxidase positive follicular melanocytes are the primary site of pigment production. Hair color basically depends on the eumelanin Abbreviations: BMD, bone mineral density; BMI, body mass index; CAD, coronary artery disease; DBP, diastolic blood pressure; FBS, fasting blood sugar; OR, odds ratio; PABA, paraamino benzoic acid; PGH, premature graying of hair; RCT, randomized controlled trials; SBP, systolic blood pressure; TERT, telomerase reverse transcriptase. Saniya Mahendiratta and Phulen Sarma have contributed equally to this study and both are considered as first authors.